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La maladie de Behçet en Tunisie. Étude clinique de 519 cas
Abstract
PurposeTo describe clinical characteristics of Behçet's disease in Tunisia.
... Behçet's disease mainly affects young men with a peculiar geographical distribution [4]. The masculinity of the disease, pathergy positivity, and venous thrombosis are remarkable in Mediterranean and Middle East countries [10]. Sub Saharan African patients had a higher frequency of neurological involvement and mortality and a lower frequency of HLA B51 allele compared with those from North Africa and Europe, respectively [4]. ...
... The prevalence is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries than in northern Europe and the USA [21]. In other African nations, the prevalence in Tunisia was 5.2/100,000 [10] and in Morocco based on registered cases adjusted for adults was estimated to be 5.1/ 100,000 [22]. In earlier attempt to study the epidemiology of BD in Senegal, 17 consecutives black patients during 26 years were reported in Dakar, Sénégal, and the majority lives in coastal areas [23]. ...
... In a previous Egyptian cohort study in 2009, the mean age of patients was 32.8 ± 8.3 years [17]. In a Tunisian study the mean age at onset was 29 [10] while in Senegal, it was 27.5 (11-42 years) with a peak onset between 20 and 30 years [23]. In Turkey, the mean age of disease onset was lower (26.2 ± 8.5 years) and the age at diagnosis was 30.4 ± 9.01 years [37] with a comparable delay in diagnosis. ...
1 2 3 Your article is protected by copyright and all rights are held exclusively by International League of Associations for Rheumatology (ILAR). This e-offprint is for personal use only and shall not be self-archived in electronic repositories. If you wish to self-archive your article, please use the accepted manuscript version for posting on your own website. You may further deposit the accepted manuscript version in any repository, provided it is only made publicly available 12 months after official publication or later and provided acknowledgement is given to the original source of publication and a link is inserted to the published article on Springer's website. The link must be accompanied by the following text: "The final publication is available at link.springer.com". Abstract Objectives The present work was conducted to estimate the prevalence of adult Behçet's disease (BD) in adult Egyptian and to study the clinical pattern and influence of age at-onset and sex on disease phenotype. Also, we investigated the spectrum of presentation and frequencies along the north-to-south gradient of the country. Patients and method The population-based, multicenter, cross-sectional study included 1526 adult BD patients from 26 specialized Egyptian rheumatology centers. Demographic, clinical, and therapeutic data are assessed for all patients. Results The mean age of patients was 35.7 ± 9.84 years, disease duration 6.58 ± 5.25 years, and age at onset 29.37 ± 8.6 years; 91 were juvenile-onset (JoBD). There were 1102 males and 424 females (M:F 2.6:1). Regarding co-morbidities, 19.92% were diabetic, and 26.05% were hypertensive. The mean body mass index was 27.57 ± 5.24 (43.1% overweight; 25.9% obese). The mean BD current activity form was 4.48 ± 4.28. Regarding the medications use, systemic steroid and colchicine were the most common drugs used (947 (90.2%) and 611 (82.7%), respectively). The overall estimated prevalence of BD in Egypt was 3.6/ 100,000 population being highest in the two main cities: Alexandria (15.27) and Cairo (8.72). Pathergy test was positive in 43.4%. 90.2% were receiving systemic steroids and 8.3%, biologics. Disease characteristics were comparable between JoBD and adult-onset BD cases. Central nervous system (CNS), deep venous thrombosis (DVT), and gastrointestinal (GIT) involvement were significantly higher in males (p = 0.01, p = 0.001, and p = 0.001 respectively) while joint affection (p = 0.001) and disease activity (p = 0.011) were increased in females. Conclusions This study provides current prevalence of BD in Egypt; 3.6/100,000 with no remarkable north-to-south gradient. The sex influences the disease phenotype with the CNS, DVT, and GIT involvement are higher in males, while the joint affection and disease activity were increased in females. Key Points • The prevalence and phenotype of Behçet's disease across Egypt is presented in a multicenter nationwide study. • The potential influence of the age at onset and sex on disease phenotype is highlightened. • A review of the literature worldwide is presented allowing comparisons with studies from other nations.
... La MB est une vascularite multi-systémique qui touche essentiellement l'adulte jeune avec des manifestations cliniques qui sont polymorphes : cutanéo-muqueuses, oculaires, gastro-intestinales, pulmonaires, vasculaires et neurologiques [3]. La prédominance masculine de notre série est retrouvée par d'autres séries maghrébines et les séries des pays du Moyen-Orient : sex-ratio entre 3 et 5 [4] contrairement au Japon et à la Turquie, où le sex-ratio est entrain de baisser avec une tendance à l'égalisation des sexes ces 20 dernières années [5]. L'âge de début de la maladie de nos patients (32.5ans) est comparable aux autres séries. ...
... Les thromboses artérielles : Les artères des membres inférieurs semblent être le siège de prédilection des occlusions artérielles [11]. D'autres localisations sont rapportées, notamment dans le territoire des artères cérébrales, des artères pulmonaires, de l'artère poplitée, de l'artère iliaque externe, de l'artère fémorale superficielle, de l'aorte sous-rénale, des artères iliaques et de l'artère rénale [4]. Ces thromboses peuvent être multiples et étagées, et sont habituellement plus fréquentes chez la femme. ...
... En effet, dans la série de Sekkach et al, 29,3% de tous les malades présentant une maladie de behçet avaient une atteinte veineuse et 90% des angiobehçet présentaient cette localisation[8]. L'atteinte veineuse est très fréquente dans le pourtour méditerranéen : 21 à 36% dans d'autres séries marocaines, 24,9% dans la série tunisienne de Hamzaoui et al et 35% en France.[4,9] Les thromboses veineuses superficielles et profondes des membres inférieurs sont de loin les plus fréquentes (52 %). ...
Introduction : L’atteinte vasculaire dans la maladie de Behçet touche 5 à 40% des patients. Tous les vaisseaux quel que soit leur type, taille ou localisation peuvent être concernés. Cette atteinte est grave pouvant engager le pronostic vital. Observations : On a colligé 12 cas de Janvier 2010 à Janvier 2017. Il s’agit de 11 hommes et 1 femme avec un âge moyen de 32,5ans. L’atteinte veineuse concernait 11 malades avec une thrombose de la veine cave supérieure (VCS) chez 6 malades, thrombophlébite des membres inférieurs chez 5 malades et une thrombose de la veine cave inférieure et des veines sus hépatiques chez 1 malade. L’atteinte artérielle touchait 9 malades (75%) avec anévrysme des artères pulmonaires chez 5 malades et une embolie pulmonaire chez 5 autres. L’atteinte cardiaque concernait la moitié des patients à type de péricardite chez 3 malades et de thrombus intra cardiaque chez 4 malades. Conclusion : L’atteinte thoracique de l’angiobehçet est fréquente et grave nécessitant un diagnostic rapide et une thérapeutique spécifique intense pour améliorer son pronostic.
... Behçet's disease mainly affects young men with a peculiar geographical distribution [4]. The masculinity of the disease, pathergy positivity, and venous thrombosis are remarkable in Mediterranean and Middle East countries [10]. Sub Saharan African patients had a higher frequency of neurological involvement and mortality and a lower frequency of HLA B51 allele compared with those from North Africa and Europe, respectively [4]. ...
... The prevalence is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries than in northern Europe and the USA [21]. In other African nations, the prevalence in Tunisia was 5.2/100,000 [10] and in Morocco based on registered cases adjusted for adults was estimated to be 5.1/ 100,000 [22]. In earlier attempt to study the epidemiology of BD in Senegal, 17 consecutives black patients during 26 years were reported in Dakar, Sénégal, and the majority lives in coastal areas [23]. ...
... In a previous Egyptian cohort study in 2009, the mean age of patients was 32.8 ± 8.3 years [17]. In a Tunisian study the mean age at onset was 29 [10] while in Senegal, it was 27.5 (11-42 years) with a peak onset between 20 and 30 years [23]. In Turkey, the mean age of disease onset was lower (26.2 ± 8.5 years) and the age at diagnosis was 30.4 ± 9.01 years [37] with a comparable delay in diagnosis. ...
Objectives
The present work was conducted to estimate the prevalence of adult Behçet’s disease (BD) in adult Egyptian and to study the clinical pattern and influence of age at-onset and sex on disease phenotype. Also, we investigated the spectrum of presentation and frequencies along the north-to-south gradient of the country.
Patients and method
The population-based, multicenter, cross-sectional study included 1526 adult BD patients from 26 specialized Egyptian rheumatology centers. Demographic, clinical, and therapeutic data are assessed for all patients.
Results
The mean age of patients was 35.7 ± 9.84 years, disease duration 6.58 ± 5.25 years, and age at onset 29.37 ± 8.6 years; 91 were juvenile-onset (JoBD). There were 1102 males and 424 females (M:F 2.6:1). Regarding co-morbidities, 19.92% were diabetic, and 26.05% were hypertensive. The mean body mass index was 27.57 ± 5.24 (43.1% overweight; 25.9% obese). The mean BD current activity form was 4.48 ± 4.28. Regarding the medications use, systemic steroid and colchicine were the most common drugs used (947 (90.2%) and 611 (82.7%), respectively). The overall estimated prevalence of BD in Egypt was 3.6/100,000 population being highest in the two main cities: Alexandria (15.27) and Cairo (8.72). Pathergy test was positive in 43.4%. 90.2% were receiving systemic steroids and 8.3%, biologics. Disease characteristics were comparable between JoBD and adult-onset BD cases. Central nervous system (CNS), deep venous thrombosis (DVT), and gastrointestinal (GIT) involvement were significantly higher in males (p = 0.01, p = 0.001, and p = 0.001 respectively) while joint affection (p = 0.001) and disease activity (p = 0.011) were increased in females.
Conclusions
This study provides current prevalence of BD in Egypt; 3.6/100,000 with no remarkable north-to-south gradient. The sex influences the disease phenotype with the CNS, DVT, and GIT involvement are higher in males, while the joint affection and disease activity were increased in females.
Key Points
• The prevalence and phenotype of Behçet’s disease across Egypt is presented in a multicenter nationwide study.
• The potential influence of the age at onset and sex on disease phenotype is highlightened.
• A review of the literature worldwide is presented allowing comparisons with studies from other nations.
... La moyenne d'âge au début de la symptomatologie de la maladie était de 27,2 ans, proche des données rapportées en Tunisie : 28,7 ans, et en Turquie : 24,2 ans [9,10]. Comme dans notre étude, la majorité des séries ont rapporté une prédominance masculine de la maladie. ...
... Comme dans notre étude, la majorité des séries ont rapporté une prédominance masculine de la maladie. Alors qu'elle était nette dans les pays du pourtour méditerranéen : 2,7 en Tunisie [10], elle était moins nette en Asie et même une légère tendance à la prédominance féminine a été constatée en Turquie : 0,73 [9], et en Chine : 0,81 [11]. ...
... Elles surviennent dans 60 à 80 % des cas et sont responsables de cécité uni-ou bilatérale si elles ne sont pas prises en charge à temps [15]. Si la fréquence de l'atteinte oculaire était comparable à celle rapportée au Maroc (67 %) [13], en Tunisie, Hamzaoui et al. ont rapporté une fréquence plus faible : 32,2 % [10]. Un patient sur deux avait une atteinte articulaire, des constatations semblables étaient décrites dans les séries tunisiennes (50 %) et marocaines (57 %) [13]. ...
Background: The aim of the current study was to describe the epidemiological and clinical features of Behc¸et disease in westernAlgeria.
Methods: This observational retrospective study examined records for patients with Behc¸et disease seen in our department from 1990 to 2015.
Results: Of the 61 patients included in the study, 80.3% were men, for a sex ratio of 4.08. The mean age at onset was 27.2�7 years, and the mean interval between the onset of symptoms and diagnosis was 4.8�6.3 years. Dermatologic manifestations were the most common finding. Oral ulcers were seen in all patients, genital ulcers in 60%, pseudofolliculitis in 56.4%, and erythema nodosum in 13.5%. A pathergy test was positive in 20% of the patients. The frequencies of the remaining manifestations were as follows: joint involvement 50%, ocular involvement 71.2%, vascular complications 29.6%, neurological involvement 19%, and gastrointestinal complications 7.5%.
Conclusion: Besides dermatological manifestations, ocular and vascular complications were the most common features observed in our patients.
... La moyenne d'âge au début de la symptomatologie de la maladie était de 27,2 ans, proche des données rapportées en Tunisie : 28,7 ans, et en Turquie : 24,2 ans [9,10]. Comme dans notre étude, la majorité des séries ont rapporté une prédominance masculine de la maladie. ...
... Comme dans notre étude, la majorité des séries ont rapporté une prédominance masculine de la maladie. Alors qu'elle était nette dans les pays du pourtour méditerranéen : 2,7 en Tunisie [10], elle était moins nette en Asie et même une légère tendance à la prédominance féminine a été constatée en Turquie : 0,73 [9], et en Chine : 0,81 [11]. ...
... Elles surviennent dans 60 à 80 % des cas et sont responsables de cécité uni-ou bilatérale si elles ne sont pas prises en charge à temps [15]. Si la fréquence de l'atteinte oculaire était comparable à celle rapportée au Maroc (67 %) [13], en Tunisie, Hamzaoui et al. ont rapporté une fréquence plus faible : 32,2 % [10]. Un patient sur deux avait une atteinte articulaire, des constatations semblables étaient décrites dans les séries tunisiennes (50 %) et marocaines (57 %) [13]. ...
The aim of the current study was to describe the epidemiological and clinical features of Behçet disease in western Algeria. This observational retrospective study examined records for patients with Behçet disease seen in our department from 1990 to 2015. Of the 61 patients included in the study, 80.3% were men, for a sex ratio of 4.08. The mean age at onset was 27.2±7 years, and the mean interval between the onset of symptoms and diagnosis was 4.8±6.3 years. Dermatologic manifestations were the most common finding. Oral ulcers were seen in all patients, genital ulcers in 60%, pseudofolliculitis in 56.4%, and erythema nodosum in 13.5%. A pathergy test was positive in 20% of the patients. The frequencies of the remaining manifestations were as follows: joint involvement 50%, ocular involvement 71.2%, vascular complications 29.6%, neurological involvement 19%, and gastrointestinal complications 7.5%. Besides dermatological manifestations, ocular and vascular complications were the most common features observed in our patients.
... She was special regarding the disease severity as she had an average of 7 recurrences per year. These results are consistent with most of the series (Table II) which show a frequency of 90-100% for oral aphthous ulcer 16,10 presenting at the beginning and in all stages of the disease. One feature of the Behçet's disease in the tropical regions is the clinical resemblance of its oral and genital sores to the ulcers due to sexually transmitted infections such as chancroid, lymphogranuloma venereum and herpes simplex virus. ...
... 18 Acneiform papules were noted in 8% of patients, which is close to the results of a Tunisian series by Hamzaoui with a frequency of 7.9%. 16 Ocular manifestations, with over 40% were the most comment extra-cutaneous abnormality in our patients. The frequency of ocular involvement is variously assessed in different studies depending on the inclusion criteria: it ranges from 29% to 100%. ...
... Our series Hamzaoui [16] Benamour [10] Oral aphtous ulcers 100% 100% 94% ...
Introduction:
Although Behçet's disease is well-documented in Eastern populations, epidemiologic data in Sub-Saharan African population is scarce. The aim of this study was to define the epidemiologic and therapeutic aspects as well as clinical course of Behçet's disease in African black population.
Results:
The study included 50 patients with Behçet's disease. The average age was 32 (18-67) years. A total of 31 patients were men and 19 were women. Two patients had a positive family history of Behçet's disease. The oral and genital aphthous lesions were present in 100% of patients. The pathergy test was positive in 16 patients (32%). Following skin conditions were observed: pseudofolliculitis in 15 patients (30%), acneiform papules in 6 patients (12%), erythema nodosum in 4 patients (8%) and leg ulcers in one patient. Ocular involvement was reported in 22 patients (44%) and joint involvement in 20 patients (40%). Neurological abnormalities were noted in 12 patients (24%). Gastrointestinal involvement with wide and deep ulcerations in the ileocecal region was observed in a patient. As treatment, a combination of oral corticosteroids and colchicine was used in 97% of our patients. Thalidomide was introduced in 3 patients and anticoagulation treatment in 19 patients. Clinical improvement was noted in 25 patients (50%), recurrence in 14 patients (28%) and 3 patients were lost to follow (6%).
Conclusion:
The Behçet's disease is not uncommon in black skin and generally affects young adults. Severe aphthous ulcers of the oral cavity and genital area are the most consistent finding.
... Several authors therefore give it great diagnostic value, which justifies its inclusion among the classification criteria for BD [1]. The positivity of this test varies between 45% and 70% of cases according to the different series published [3][4][5]12]. The dispersion of the results can be explained by the ethnic origin of the patients, the heterogeneity of the techniques for carrying out the pathergy test between the teams and the taking of colchicine or anti-inflammatory drugs before performing this test. ...
Behçet's disease is a chronic inflammatory disease characterized by its clinical polymorphism associating mucocutaneous involvement to systemic manifestations. The mucocutaneous lesions are considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Our objective was to determine the characteristics of this skin involvement during Behçet's disease. We conducted a descriptive study over a period of 30 years, having collected all patients with Behçet's disease. These were 98 patients. A male predominance was observed in our studied population with a Sex Ratio of 2.5. The mean age at diagnosis was 34 years. Mucocutaneous involvement was observed in all patients. Oral aphthosis was constant and genital ulcers, were observed in 81 cases. The other mucocutaneous manifestations were: pseudofolliculitis (61 cases), erythema nodosum (7 cases), skin ulcers (4 cases), acneiform lesions (2 cases), perianal ulcers (1 case), skin ulceration (1 case) and erythema multiforme. (1 case). All of our patients were treated with colchicine. Corticosteroids and non-steroidal anti-inflammatory drugs were each indicated in one case for resistant forms.
... The prevalence of CNS involvement varies widely from 1.3% to 59% depending on diagnosis criteria and ethnic populations (4,5). Epidemiological studies show that BD is the most frequent vasculitis in Tunisia (6) and the frequency of NBD is 28.1% (7). It appears about 5 years after the onset of BD symptoms. ...
Background: When the central nervous system (CNS) is the primary affected site in an initial attack of Behcet’s disease (BD), the differential diagnosis is particularly challenging. Some cases remain unclassified or qualified as probable Neuro-Behçet disease (NBD). Moreover, it was demonstrated that cytokines play a crucial role in the pathogenesis of NBD. We therefore studied peripheral and cerebrospinal inflammatory profile of these patients.
Methods: Twenty two parenchymal NBD patients diagnosed according to the international consensus recommendation criteria and classified into definite (d-NBD; n= 13) and probable (p-NBD ; n=9) were sampled at their first neurological symptoms and compared with healthy control subjects (n=10). Oligoclonal bands of IgG were detected by isoelectric focusing on agarose and immunoblotting of matched serum and Cerebrospinal fluid (CSF) sample pairs. Cytokines and transcription factors related to TH1, TH2, TH17 and T regulatory populations were studied by quantitative RT-PCR in the CSF.
Results: Oligoclonal bands (OCB) were present in only 1/22 patients. Two d-NBD patients had OCB in the CSF showing pattern 4. In NBD CSF samples, INF gamma, IL-17 and IL-10 expressions were significantly elevated compared with controls, however no difference in those cytokine expressions was observed between d-NBD compared to p-NBD. The most stricking finding was the significant increase of CSF IL-6 in d-NBD compared to p-NBD.
Conclusion:These results indicate the rare presence of OCB in parenchymal NBD patients. Additionally, CSF IL-6 could help us to identify definite NBD.
Keywords: Behçet’s disease, central nervous system, cerebrospinal fluid, cytokine.
Background:
Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial.
Objectives:
The primary aim of our study is to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study is to compare the data of newly diagnosed patients registered between 1998-2020 with the data of those registered between 1976-1997.
Methods:
Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of ICBD at or before 16 years of age.
Results:
A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976- 1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement.
Conclusions:
Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.
When the central nervous system (CNS) is the primary affected site in an initial attack of Behçet’s disease (BD), the differential diagnosis is particularly challenging. Some cases remain unclassified or qualified as probable neuro-Behçet’s disease (NBD). Several cytokines are involved in the immunopathogenesis of this disease; however, studies establishing the differential cytokine pattern between probable and definite NBD are scarce. Twenty-eight parenchymal NBD patients, diagnosed according to the International Consensus Recommendation (ICR) criteria and classified into definite (D-NBD; n = 17) and probable (P-NBD; n = 11), were sampled at their first neurological symptoms, and compared with healthy control subjects (n = 20). Oligoclonal bands (OCB) of IgG were detected by isoelectric focusing on agarose, and immunoblotting of matched serum and cerebrospinal fluid (CSF) sample pairs. T cell cytokines (INF-γ, IL-4, IL-17, and IL-10) and transcription factors related to Th1, Th2, Th17, and T regulatory populations (respectively T-bet, GATA-3, ROR-γt, and Foxp3) were studied by quantitative RT-PCR in peripheral blood mononuclear cells (PBMCs) and CSF cells. Inflammatory cytokines such as IL-6, TNF-α, and IL-1β were also analyzed. CSF OCB pattern 2 was present in only 1 out of 28 neuro-Behçet’s patients who belonged to the P-NBD group. Two D-NBD patients had OCB in CSF showing pattern 4. In the D-NBD CSF samples, IL-17 and IL-10 expressions were significantly elevated compared to P-NBD. Moreover, D-NBD patients had increased levels of T-bet/GATA-3 and ROR-γt/Foxp3 ratios compared to P-NBD. Furthermore, a significant increase of CSF IL-6 in D-NBD, compared to P-NBD and the controls, was found. In addition to the increased IL-6 level, the data obtained suggest the existence in D-NBD patients of a significantly disrupted balance between Th17 effector and T regulatory cells, as reflected by the enhanced ROR-γt/Foxp3 ratio. This could be considered as an additional criterion for definite neuro-Behçet’s disease.
The aim was to investigate the frequency and spectrum of cardiac involvement (CI) in patients with Behçet syndrome (BS) in the Tunisian context, and to assess the clinical and imaging features, treatment, and outcomes. We retrospectively retrieved the medical records of patients with CI among 220 BS patients admitted to the hospital internal medicine department between February 2006 and April 2019, who fulfilled the International Study Group diagnostic criteria for BS. Ten patients (8 men, 2 women) were eligible for the study. Mean age was 37.3 years. Three patients had 2 isolated episodes of cardiac BS. The different types of CI were coronary artery disease (5/10), intracardiac thrombus (4/10), pericarditis (1/10), myocarditis (1/10), and myocardial fibrosis (1/10). Five patients had associated vascular involvement (50%). Medical treatment was based on corticosteroids and colchicine in all patients (100%), anticoagulants in 8 (80%), and cyclophosphamide followed by azathioprine in 9 (90%). The clinical course was favorable in 9 patients; 1 patient died. CI remains an important feature of BS because of its association with increased risk of mortality and morbidity. Therefore, early screening and detection with imaging methods are paramount. Also, better cooperation between rheumatologists and cardiologists could improve outcomes.
Résumé
Introduction
La maladie de Behçet est une affection inflammatoire systémique. L’atteinte oculaire souvent au premier plan constitue un critère diagnostique important. Elle est fréquente et grave pouvant mettre en jeu le pronostic visuel.
BUT
Étudier les caractéristiques épidémiologiques, cliniques et thérapeutiques des manifestations oculaires de la maladie de Behçet et de rechercher des facteurs prédictifs d’atteinte oculaire sévère.
Méthodes
Étude rétrospective de 93 observations d’oculo-Behçet sur une période de 9 ans. Les données ont été analysées rétrospectivement à partir des dossiers médicaux. Une atteinte oculaire sévère était définie par une acuité visuelle limitée à une perception lumineuse négative ou mal orientée.
Résultats
Le sex ratio H/F était de 1,9. L’âge moyen était de 34,5 ans. L’atteinte oculaire était révélatrice de la maladie dans 4%, bilatérale dans 61% et active dans 68,8%. L’uvéite était l’atteinte la plus fréquente (57%) dominée par la panuvéite, suivie par la vascularite rétinienne (51,6%) et l’œdème papillaire (10,7%).
La maculopathie (26,8%) et la cataracte (18,2%) représentaient les complications les plus fréquentes. Vingt malades (21,5%) avaient une perception lumineuse négative ou mal orientée.
Le traitement par corticothérapie par voie générale en association avec les immunosuppresseurs (Azathioprine) était prescrit dans 55,9%. Le sexe masculin et la panuvéite étaient des facteurs prédictifs d’atteinte oculaire sévère
Conclusion
L’atteinte oculaire au cours de la maladie de Behçet est fréquente et grave pouvant aboutir à la cécité. Le sexe masculin et la panuvéite constituaient des facteurs de mauvais pronostic visuel.
Aim of the work
To determine the frequency of initial ocular findings and subsequent ocular complications among a cohort of Behçets disease (BD) patients attending the outpatient clinic of a tertiary hospital.
Patients and methods
The medical records of 94 BD patients attending the Rhuematology and Ophthalmology outpatient clinics of Ain Shams University Hospitals were retrospectively reviewed. The frequency of ocular findings and subsequent complications were recorded.
Results
The mean age of patients at diagnosis was 37 ± 12 years; 65 were males and 29 females (M:F 2.24:1); 93.6% resided in urban and 6.38% in rural areas. Current management regimen included oral steroids in 60 patients, with pulse steroids given in case of disease activity. 32 patients received azathioprine and 48 patients received colchicine. Ocular manifestations were the presenting complaint in 7 (7.4%) patients; 5 complaining of redness, and 2 photophobia and excessive lacrimation, and 95.7% (n = 90) had ocular findings later on. The mean intraocular pressure was 13.8 ± 2.5 mmHg in the right eye and 15.5 ± 2.6 mmHg in the left. Anterior uveitis was present in 41.5%, posterior in 26.6% amd pan uveitis in 28.7%. 3 had a lost eye, 10 cataract, 6 glucoma and others (n = 5) had macular oedema and phthisis bulbi. Visual acuity was 6/12 in 64 (68.1%).
Conclusion
Ocular manifestations were the presenting complaint in fewer than 10% of patients, and subsequent frequency of ocular complications reported in our study is among the highest recorded. Further multicenter studies are needed to identify ocular complications in BD.
Behçet syndrome (BS) is more prevalent along the ancient trading route known as the “Silk Road,” extending from the Mediterranean to the countries in the Far East, where the prevalence of HLA-B5 (51) is relatively more frequent in the healthy population. This suggests that the possible causative agent(s), including genetic factors such as HLA-B51, might have had spread along this route. Seven cross-sectional and population-based field studies showed that the prevalence of BS was between 19 and 421 among 100,000 adolescent/adult population in Turkey. The lower three frequencies (19, 20, and 80/10⁵) come from Thrace, the European part. The disease is less common in Western Europe, the USA, and in the rest of the world but perhaps not as rare as it had previously been thought. A recent study showed that BS was as frequent (7.1/10⁵) as other vasculitic diseases – 9/10⁵ for the combination of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome – in a suburb of Paris, France. This chapter focuses on the epidemiological features of the field surveys of BS in Turkey and the rest of the globe as well as regional differences in some of the clinical and laboratory features. Gastrointestinal disease is frequent in patients from the Far East (especially Japan), while it is rather infrequent among those from Turkey. Moreover, a high frequency of both pathergy test positivity and being a carrier of HLA-B5 (51) is observed in patients from Japan and the Mediterranean countries, in contrast to the patients from the UK and the USA.
Joint involvement in Behçet syndrome (BS) occurs in half of the patients in the form of arthritis or arthralgia. Behçet himself described joint involvement in 1938, a year after the original description of the disease. It is usually mono- or oligoarticular and can be symmetrical when not monoarticular. The arthritis usually lasts a few weeks, and it seldom results in deformity and radiological erosions. Chronic arthritis is seen only occasionally. The most frequently involved joints are the knees, followed by ankles, wrists, elbows, and hands. Axial involvement in the form of spinal, shoulder, hip, and sacroiliac joints is rare. In addition, back pain is quite rare and controlled studies have not shown an increased sacroiliac joint involvement. Patients with BS and arthritis also have more acne lesions. Furthermore, patients with arthritis and acne lesions have significantly higher enthesopathy scores than patients without acne. This suggests a possible pathogenetic link with acne-associated arthritis. Laboratory features are generally nonspecific. The erythrocyte sedimentation rate and C-reactive protein may be mildly elevated, but these may not correlate with the severity of arthritic attacks. Autoantibodies like rheumatoid factor and antinuclear antibodies are absent. Synovial fluid is inflammatory with a predominance of polymorphonuclear leucocytes although it has a good mucin clot. Synovial histology shows nonspecific inflammation. Fibromyalgia can be associated with BS especially among the female patients. Generalized or localized myositis and osteonecrosis are infrequently seen.
Background:
Cardiac and vascular involvement in Behçet disease (BD), also referred as vasculo BD, is frequent. We aimed to describe clinical characteristics, predictive factors and management of vasculo BD in the Tunisian context.
Methods:
We retrospectively studied 213 records of all BD patients followed between January 2004 and May 2016 in the Internal Medicine Department and who fulfilled the ISGBD criteria. We described first clinical features of BD with cardiac and vascular involvement then predictive factors were studied in univariate then multivariate analysis.
Results:
Among the 213 patients, 64 (30%) were diagnosed as having vasculo BD. The mean age at diagnosis was 31.5 years. About 81.25% of them were males and 18.75% females. Vascular involvement associated or not with cardiac involvement was found in 64 patients (30%). Deep venous thromboses are most common (62.5%) compared with superficial ones (23.4%), pulmonary arterial thrombosis (14.1%) or aneurysms (9.4%). Cardiac involvement is ranging from pericarditis (1.6%) to intra cardiac thrombosis (3.1%) and myocardial infarction (1.6%). Predictive factors associated with cardiac and vascular involvement in BD are male gender (OR = 3.043, 95% CI = 1.436-6.447, p = 0.004), erythema nodosum (OR = 4.134, 95% CI = 1.541-11.091, p = 0.005) and neurologic involvement (OR = 2.46, 95% CI = 1.02-5.89, p = 0.043).
Conclusion:
Cardiac and vascular involvement in BD is frequent in the Tunisian context with a broad spectrum of manifestations ranging from vascular involvement to cardiac one. Male gender, patients with erythema nodosum or neurologic involvement are prone to develop cardiac or vascular features of BD needing therefore a close monitoring.
Context: This systematic review and meta-analysis was performed to determine the prevalence (by overall relative frequency) and
clinical information of the Behcet’s disease in Middle East and north Africa (MENA) and it has an important effect on the health
policy and performing complementary studies in future.
Evidence Acquisition: We performed this systematic literature review from several databases including PubMed, Scopus and ISI
Web of Science according to search strategy with two filters, place (MENA countries) and time (all articles published from Jan 1980
to Jan 2016 were considered). The keywords such as “Behcet’s syndrome”, “Behcet’s disease”, “triple symptom complex”, “Middle
East” were searched. Out of 4013 relevant articles and according to inclusion and exclusion criteria, 28 papers were selected to study.
To examine the quality of the studies, all papers were evaluated independently by two authors and the Kappa coefficient was 95%.
Afterwards, the critical appraisal was performed by strengthening the reporting of observational studies in epidemiology (STROBE)
form. Due to sever heterogeneity, the pooled prevalence (per 100,000 people) was derived by the random effect model that takes
between-study variation into account.
Results: According to the results of the present study, the prevalence rate of BD in Iran was 68; it was after Turkey among MENA. The
pooled prevalence (per 100,000 people) of BD in MENA was 120 (95% CI: 86.8, 166.8) according to the random effect model. The oral
aphthosis was the most frequent symptom among other symptoms and HLA-51 association was its poor prognosis. Turkey had the
highest prevalence (per 100,000 people) of 420 (95%CI: 340, 510) and the lowest prevalence 2.1 related to Kuwait in MENA countries.
Conclusions: There are different statistics about the prevalence rates of the disease that have been published; these different prevalence
statistics can be explained by racial and geographical divergence, patient selection and BD diagnosis criteria. The results of
this study can be useful for health policy and other studies which are needed to find the reasons of this prevalence difference.
The behcet disease is a vasculitis with polyorganic attacks whose diagnosis is established by
the international study group for behcet disease (ISGBD).
The arterial ulcer or aphta has no diagnostic value in the criteria of ISGBD.
its morphological similarity and most likely pathophysiological also, with bipolar ulcers
deserves attention.
This article is an observation relating the history of a patient who does not respond to criteria
of ISGBD despite the presence of an arterial aphta.
The tradive occurrence of minor criterion was a cause of diagnostic and therapeutic delay.
Behçet's disease (BD) is a systemic affection with unclear cause and pathogenesis. The disease is characterized by great clinics polymorphisms with a particular frequency of the dermatological manifestations which account for three of the four criteria of the disease's classification adopted by the International Study Group (ISG) (table 1). Vascular involvement, called also, «angio-Behçet» or «vasculo-Behçet» is particular because, it's observed in young men without thrombotic or cardiovascular risk factors. All the vessels, arterial or venous, whatever their localization can be affected, with often, multifocal vascular manifestations. Vascular involvement is a severe disease, regarding the vital prognosis; it justifies a fast and aggressive therapeutic management. Venous involvement recovers 80 to 90% of the vascular manifestations; it is found in a third of the patients. It is described at all the ages, and occurs in more half of the cases during the first five years of the evolution of the disease. This manifestation can precede or can be the mode of entry in the disease in meadows of 10% of the cases. Deep venous thrombosis of the legs is the most prevalent and represents 60 to 70% of venous localizations of the disease. It is often bilateral. Superficial venous thrombosis is also frequent and is mostly confused with an erythema nodosum. The involvement of large venous vessels is prevalent. Veina cava thrombosis is the second localization of the venous involvement during BD and represents nearly a quarter of the thrombotic manifestations. Chiari syndrome and/or portal thrombosis are associated with bad prognosis. Cerebral venous thrombosis are reported in 5 to 10% of BD. Intracardiac thrombosis are extremely rare but are recognized like a classical complication of the BD. Venous thrombosis during BD have a less embolic character than the idiopathic thrombophlebitis, because of wall inflammatory phenomenon who makes the thrombus more adherent. However, pulmonary embolisms are reported in 10 to 15% of cases and are associated with pulmonary artery anevrysms or with right intracardial thrombosis. Therapeutic management of this manifestation does not rise from randomized or comparative studies. They are generally retrospective and observational studies. It gets clear however clearly that these cases of thrombosis must profit from a treatment aiming to reduce the wall inflammation. Corticosteroids and immunosuppressant allows reducing quickly the clinical symptoms and recurrences. Anticoagulation therapy is usually proposed but the benefit of this treatment is not demonstrated and the recent EULAR guidelines of the management of BD do not recommend the systematic use of anticoagulants in these cases.
The new edition of this highly successful volume continues to offer readers with a systemized and objective approach to the practice of oral and maxillofacial medicine. Winner of the Royal Society of Medicine and Society of Authors Book Award in the category of New Authored Book (2004), and Highly Commended in the British Medical Association Book Awards (2009), this volume is ideal for senior dental students, dental practitioners and for trainees and practitioners in oral medicine, surery, and pathology in particular.
Aim:
To estimate the distribution of HLA Class I (A, B) antigens in patients with Behcet's disease (BD) and the association of HLA-B5 antigen with the clinical manifestations of the disease in different ethnic and population groups in relation to gender.
Subjects and methods:
The study covered 93 patients (68 males, 25 females) from the representatives of 24 ethnicities with the verified disease. HLA Class I antigens were typed by the microlymphocytotoxic technique, by applying an antileukocytic serum kit (GISANS, Saint Petersburg).
Results:
In patients with BD, the prevalence of HLA-B5 antigen proved to be significantly higher than that in the controls (72.0 and 21%, respectively) and to be similar in patients of different ethnicities living in the Caucasus and Transcaucasus (80-83%) while the number of HLA-B5 antigen-positive patients with BD was thrice less in the Russian population than in other BD patients (p < 0.01). There was a significant correlation of HLA-B5 antigen with ocular lesion (retinal angiitis) predominantly in male patients with BD.
Conclusion:
The prevalence of HLA-B5 antigen was higher in patients with BD than in the population-based control. The diagnostic value of this antigen is not so great, for example, in the Russian population of patients with BD. The presence of HLA-B5 antigen in the phenotype of male patients with BD may be regarded as a prognostically poor marker of development of eye diseases.
Purpose: To investigate and compare the causes of blindness in ocular Behçet's disease (BD) in men and women Methods: In a retrospective, descriptive investigation from 1976 to 2008, 6,021 BD cases were registered in our BD Unit of Shariati Hospital of Tehran University of Medical Sciences (TUMS). At the last visit, 187 patients (124 men and 63 women) were blind (vision=1/10 or less) at least in one eye and with at least 3 years of follow-up in our clinic. All patients received conventional treatments for BD following the diagnosis. Results: 187 unilateral or bilateral blind cases of BD, 124 males (244 eyes) and 63 females (124 eyes) were included in our study. They were blind (VA=1/10 or less) at the last visit. The mean age of men was 31.74±8.6 years, the mean age of women was 33.13±10.26 years at presentation, t=0.97, P=0.3. At presentation 229 eyes (62.23%) had severely impaired vision (VA≤1/10), 144 eyes (59.02%) of men and 85 eyes (68.55%) of women, χ2 =0.403, P=0.5. The mean duration of diagnosis up to 2008 was 13.85±6.42 years in men and 15.65±6.41 years in women, t=1.8, P=0.05. The end-blinding outcome was registered in 77.99% (N=287) eyes, 78.28% (N=191) eyes in men and 77.42% (N=96) eyes in women. χ2 =0.05, P=0.8. The most common cause for blindness was end-stage disease (retinal vascular necrosis or fibrosis, chorioretinal and optic atrophy) which was observed in 39.67% (N=146) eyes, 40.98% (N=100) eyes of men and 37.09% (N=46) eyes of women, χ2-0.163, P=0.7. Conclusion: BD can have a very severe and blinding outcome, but the end blinding result does not seem to be different in two genders.
IntroductionThis is an analysis of adult Behcet's disease (BD) in Iran, from the Iran Registry of 7187 BD patients, gathered from 1975 to 2014, among which 6075 were adults (84.5%).PatientsPatients were seen by a multidisciplinary team of experts. The diagnosis was by expert opinion, not by any specific classification/diagnosis criteria. However, 96.8% of them were classified by the International Criteria for Behcet's Disease (ICBD). Adult BDs were patients who had their first manifestation at the age of 16 or later.ResultsMales constituted 56% (male/female ratio 1.3/1). The mean age at onset was 28.3 ± 8.7, mean duration 10.8 ± 8.2, and mean follow-up 5.0 ± 6.3. Oral aphthosis was seen in 97.5%, genital aphthosis 65.7%, skin manifestations 64.6% (pseudofolliculitis 53.2%, erythema nodosum 23.9%), ocular manifestations 58.1% (anterior uveitis 41.1%, posterior uveitis 45%, retinal vasculitis 33.6%, cataract 24.4%), joint manifestations 39.4% (arthralgia 18.9%, monoarthritis 9.1%, oligoarthritis 17.8%, ankyloing spondylitis 2%), gastrointestinal manifestations 7% (gastroduodenitis 2.3%, peptic ulcer 1.2%, diarrhea 2.1%, rectorrhagia 1.0%, abdominal pain-nausea 1.8%), neurological manifestations 10.6% (central 3.7%, peripheral 0.3%, headache 7.6%), vessel involvement (large vessel 1.7% with large vein thrombosis 1.1% and arterial involvement 0.7%, phlebitis 6.6%, superficial phlebitis 2.3%), epididymitis 4.6%, pulmonary manifestations 1% and cardiac manifestations 0.6%. Positive pathergy test was seen in 52.3%, human leukocyte antigen (HLA)-B5 in 54%, HLA-B51 in 48.9%, and high erythrocyte sedimentation rate in 52.8% of patients. By International Study Group (ISG) criteria 77.9% were classified, compared to ICBD revised criteria with 96.9%. The specificity of ISG was 99.2% and ICBD 97.2%.Conclusion
Results are near the nationwide surveys from Japan, China, Korea and Germany.
Purpose: To investigate long-term ocular manifestations of ocular Behcet's disease (BD), and the causes for blindness Methods: In August 2008, at Shariati hospital of Tehran (Behcet unit) all files of the ocular BD patients from 1976 to 2008 who were legally blind (Visual acuity=20/200 or less) at least in one eye at the last visit and had at least three years of follow-up were reviewed and the desired informations were extracted. In this retrospective, comparative, investigation, 187 patients (374 eyes) were included in this group (Group 1). They were compared with 81 nonblind (at the last visit) ocular BD patients (162 eyes) who were visited and consequently selected in 2008, our control group (Group 2). They were matched in term of the duration of BD, approximately 18 years. All patients had conventional treatments for ocular BD. Results: 65.2% (N=122) of the blind group were males vs. 56.8% (N=46) of the nonblind (control group), χ2=1.73, P=0.189. High number of initially impaired vision at presentation (vision of 20/200 or less) in the blind group; 230 eyes (61.5%) vs. 18 eyes (11.2%) in the control group was highly significant, χ2=115.6, P=0.000. More frequent cases of visible retinal vasculitis (by ophthalmoscopy) in the blind group; 290 eyes (77.5%) vs. 107 eyes (66.5%) in the control group, χ2=7.78, P=0.005, and higher frequency of vitritis and/or anterior uveitis 90.9% (N=340 eyes) vs. 75.3% (N=122 eyes) in the control group, χ2=23.01, P=0.000 were both considered two major blinding risk factors in ocular BD. At the last visit 77.3% (N=289) eyes in the main group were blind and the main cause of blindness was end-stage disease (retinal vascular necrosis and consequently chorioretinal atrophy and optic atrophy) in 38.5% (144 eyes), and the second major blinding cause was macular scar ± optic atrophy in 14.9% (N=56 eyes). Conclusion: Although ocular BD can have a very severe and blinding outcome, early detection and prompt and intensive treatments may control the disease and save the sight.
Behçet’s disease is a chronic vasculitis clinically characterized by relapsing orogenital aphthosis associated with a large multisystemic spectrum of clinical manifestations. Imaging is essentially helpful to evaluate vascular involvement and to monitor post-therapeutic evolution of cardiovascular, neurological, respiratory, and digestive involvements. Familiarity with the various and specific imaging findings of this disease is fundamental to make an early detection and guide the optimal care of patients.
This case illustrates a very rare localization of false aneurysm in Behçet's disease and demonstrates the importance of monitoring treated patients.
The diagnosis of severe Behçet's disease was established in a young man after discovery of a cerebral venous thrombosis. One year later, the patient required emergency surgery for a symptomatic pseudoaneurysm of the internal iliac artery.
Performed after a 3-day regimen of corticosteroid boluses, the pseudoaneurysm was treated by ligation of the hypogastric artery. Corticosteroids were then given for immunosuppression.
Arterial involvement in Behçet's disease can be at the forefront of the clinical features and can cause potentially fatal complications. False aneurysm of the internal iliac artery is exceptional with only two cases reported in the literature. These false aneurysms occur on a fragile artery wall. Treatment requires the administration of immunosuppressive drugs.
This rare clinical presentation highlights the importance of clinical and radiological monitoring in young patients with Behçet's disease.
Copyright © 2015 Elsevier Masson SAS. All rights reserved.
Behçet's disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.
Copyright © 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Behçet's disease is a multisystemic inflammatory disease characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The diagnosis of Behçet's disease is based on clinical criteria. The etiology of the disease is unknown but the wide variations of ethnic prevalence and of the prevalence in the same ethnic group in different geographic areas indicate environmental triggering of a genetically determined disorder.
A retrospective analysis of the medical charts of 150 Behçet's disease patients seen in our internal medicine department between 1995 and 2010 was undertaken. Patients with confirmed ocular involvement were analyzed and compared with those without ocular involvement.
Among the 150 medical charts studied, 85 patients were included in the study. Thirty-three patients (38.5%) had ocular involvement. Mean age at ocular BD diagnosis onset were 35.3. Male to female ratio was 5.6. Ocular involvement was bilateral in 26 patients (78.8%). Uveitis was the most common ocular lesion (n=31 patients, 93.9%). Panuveitis was the most common anatomical location (n=21, 63.6%). The comparison of patients treated for BD with or without ocular involvement showed a statistically significant association between ocular and neurological manifestations (p=0.03). All patients with ocular involvement were treated with corticosteroids. Immunosuppressive (IS) treatments were used in 28 patients (84.8%). Cyclophosphamide was the most used as first-line treatment (71.4%). Cyclophosphamide relayed by azathioprine was the most adopted protocol (28.5%). In case of resistance or relapse and depending on the other manifestations of the BD, the IS used in first intention was replaced by another one. Seven of the 33 patients had received treatment with infliximab (IFX) after failure of other therapeutic lines.
Ocular prognosis in the BD can be improved by early treatment and regular monitoring. It is important to adjust the therapeutic protocol to the anatomic form, to the severity of uveitis and to the extra-ocular manifestations associated.
Copyright © 2014 Elsevier Masson SAS. All rights reserved.
AimBehcet's disease (BD) is a multisystem inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers and uveitis. Demographic and clinical features of BD are different in various countries. Due to these ethnic discrepancies, we decided to consider the clinical picture of BD in the Azeri population of Iran and compare it with other ethnic groups.Methods
This cross-sectional cohort study was carried out at the Connective Tissue Diseases Research Center of Tabriz University of Medical Sciences, Tabriz, Iran from 2006 to 2013. We considered the demographic and clinical findings in 166 patients with BD. Disease activity was measured by the Iranian Behcet's Disease Dynamic Activity Measure (IBDDAM) and Total Inflammatory Activity Index (TIAI).ResultsThe male-to-female ratio was 1.7 : 1.0; the age of disease onset was 25.8 ± 8.9 years. Recurrent oral aphthous ulcers were the initial manifestations of BD in 83.1% of patients. Panophthalmitis and panuveitis were the most common ophthalmic manifestations of disease. Blindness occurred in 7.1% of patients. This study showed no difference between the two genders in mean age of disease onset and clinical manifestations. However, IBDDAM in men was higher than women. Retinal vasculitis in men was more common than women.ConclusionsBD in the Azeri population of Iran starts in the third decade and has a male predominance. The activity of the disease and retinal vasculitis in men is more predominant than women in Azerbaijan.
Objective:
We investigated the effect of gender on the clinical Adamantiades-Behçet's disease (ABD) phenotype with data from the German ABD registry and a meta-analysis from a systematic literature review.
Methods:
Using the German ABD registry data, we compared 36 clinical variables by gender (with women as the reference category) and investigated potential effect modification by HLA-B5 or ethnic background. The registry data were combined with those from a literature search to calculate pooled relative risks (RRs) for variables with data from ≥10 relevant datasets.
Results:
The German ABD registry provided information for 747 subjects (58.1% males) and the systematic literature review identified another 52 datasets informing on 16 variables. Both analyses consistently revealed the association of male gender with ocular involvement (RR 1.28 and 1.34 from the ABD registry and meta-analysis, respectively), folliculitis (RR 1.30 and 1.26), papulopustular lesions (RR 1.23 and 1.25), vascular involvement (RR 2.31 and 2.27), superficial (RR 2.96 and 1.63) and deep venous thromboses (RR 2.56 and 2.16) and female gender with genital ulcers (RR 0.78 and 0.92) and joint involvement (RR 0.79 and 0.89). The ABD registry data additionally showed male gender associated with heart involvement (RR 10.60), whereas the meta-analyses revealed male gender associated with the pathergy test (RR 1.14) and female gender associated with erythema nodosum (RR 0.86). HLA-B5 and Turkish or German origin did not affect the observed associations.
Conclusion:
These analyses support gender-associated clinical variations in ABD and in particular a clinically meaningful risk of cardiovascular involvement for men.
Purpose
To identify the epidemiological characteristics and the most common etiologies of uveitis in the Tunis area.
Methods
Medical records for all uveitis patients seen from September 2003 through October 2009 were included.
Results
A total of 424 patients (596 eyes) were included. The mean age at onset of uveitis was 36 years, and the male-to-female ratio was 0.66. Uveitis was unilateral in 56.4%. Anterior uveitis was most common (48%), followed by panuveitis (33.6%), posterior uveitis (13.3%), and intermediate uveitis (5%). The most common causes were Behçet disease (14.7%), toxoplasmosis (10.2%), Vogt-Koyanagi-Harada (VKH) syndrome (3.7%) and sarcoidosis (3.3%). Retinal vasculitis was found in 20%. Behçet disease was the most common cause of chronic uveitis. The most common complications were cataract (21.6%), ocular hypertension (12%) and macular edema (5.6%).
Conclusion
In our study, the most common causes of uveitis were Behçet disease, toxoplasmosis, VKH syndrome and sarcoidosis.
Objectives:
In current study we evaluated clinical features of Behcet's Disease (BD) in patients without oral aphthosis (NOA cases).
Methods:
In a cohort of BD, patients registered during a period of 36 years were collected. We determined clinical features of BD NOA cases and compared them with patients with oral aphthosis (OA cases). The comparison was performed by chi square and Fischer's exact test.
Results:
Among 6,821 BD patients, 175 patients (2.56%) were NOA cases. Male/Female ratio was less in NOA cases (p-value: 0.078). Mean age of disease onset was significantly higher in NOA cases (p-value: 0.001). Among NOA cases, the first manifestations comprised uveitis (70.3%), joint involvement (8.0%), retinal vasculitis (6.9%), and genital aphthosis (4.0%). During the course of disease, the prevalence of ocular lesions and positive pathergy test were significantly higher in NOA cases. Conversely genital aphthosis (OR: 0.048), mucocutaneous (OR: 0.470), joint involvement (OR: 0.478), and positive family history for BD (OR:0.138) were significantly less frequent in NOA cases. NOA cases fulfilled different criteria including International Criteria for BD (ICBD), Japan Revised, Iran, Dilsen, and Classification Tree.
Conclusions:
These results addressed the distinct clinical features in NOA subset of BD Including more prevalent eye involvement and positive pathergy.
PurposeTo study antigen HLA class I association with different clinical forms of Behçet's disease in South Tunisian population.Patients and methodsWe retrospectively reviewed 129 clinical case patients. All of the patients fulfilled the criteria of the international study group for Behçet's disease, and were followed at the department of internal medicine of the university hospital of Sfax. HLA class I phenotyping was performed by microlymphocytotoxicity complement dependent for our 129 patients and for 123 healthy controls. We used the program SPSS 11.0 to analyse clinical data and to compare HLA class I antigen distribution between these two populations.ResultsThe study group concerned a total of 129 patients (81 males and 48 females). The mean age at disease onset was of 32 years. HLA-B51 antigen was the only antigen significantly more frequent among patients (24.81%) than controls (9.76%, p = 0.002). HLA-B44 was significantly more frequent among patients having familial history of recurrent buccal aphthosis or Behçet disease. HLA-A11 antigen was associated with early disease onset, and HLA-A1 was negatively associated with severe form of the disease (neurological, vascular or ocular manifestations).Conclusion
Our study confirmed the HLA-B51 association with Behçet disease. Nevertheless, B51 frequency in South Tunisian patients was lower than that found in other studies regardless of the clinical manifestation.
Background
Behçet's disease is recognized as a multisystem disorder that affects mainly young adults in Mediterranean, Middle Eastern, and Far Eastern countries. The diagnosis is very difficult because there is no laboratory test. Clinical features – such as orogenital aphtae, ocular and skin lesions, arthritis, and neurologic, gastrointestinal, vascular, and pulmonary symptoms – are helpful for diagnosis. Various cardiovascular manifestations, such as pancarditis, acute myocardial infarction, conduction system disturbances, and valvular diseases, have been reported but are rare. Intracardiac thrombus formation, as seen in our patients, is exceptional even among cardiovascular cases of Behçet's.
Observations
We report three cases of intracardiac thrombosis among 204 patients followed for Behçet's disease within our unit over a period of 7 years. We report outcomes after corticosteroid, cyclophosphamide, and oral anticoagulant therapy.
Discussion
Cardiovascular involvement has been reported in 7 % to 29 % of patients with Behçet's syndrome. Intracardiac thrombosis is extremely rare and the right heart is the most common site of involvement. The first symptoms and signs of the disease frequently precede systemic organ manifestations.
Conclusion
Diagnosis of Behçet's disease might be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical features of the condition. This is particularly applicable if the patient is a young male from the Mediterranean basin or the Middle East. We suggest that the treatment could include colchicine, anticoagulant therapy, and corticosteroids and discuss immunosuppressive therapy.
Introduction:
Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations.
Methods:
We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required). Data were retrieved and analysed with two softwares (Access(®) and Epi Info(®)).
Results:
We observed 30 cases with vascular lesions on a series of 92 patients with Behcet's disease. Most patients were male, with an average age around 40. The venous manifestations, concerning essentially the lower limbs (deep and superficial thrombosis) were found at 27 patients (90 %), and the average of age during the appearance of the venous lesions was 40 years. Arterial lesions appear more late in 13 patients (43 %) (average of age 43 years). We noted, on the other hand, 11 cases of aneurysms and five cases of arterial thrombosis. The use of corticosteroids was necessary in all cases in association with the others drugs (anticoagulants, colchicine, immunosuppressors). Among the patients having had aneurysms, six were treated surgically. The outcome was favorable for most patients. Two patients had pulmonary embolism and two post-surgery complications. One patient died in the consequences of an intragastric break of an aneurysm of the abdominal aorta.
Conclusion:
The vascular involvement in Behcet's disease is manifested primarily by thrombophlebitis. Achieving blood pressure, less common, is problematic therapeutic because of the recurrent and life threatening.
Nous rapportons l’observation d’un patient de 69 ans pris en charge en réanimation pour un tableau de méningoencéphalite à formule panachée. Dans un premier temps, la suspicion d’une étiologie infectieuse a conduit à introduire un traitement anti-infectieux probabiliste. La découverte durant l’hospitalisation d’une maladie de Behçet a élargi la prise en charge aux étiologies non infectieuses. Une corticothérapie à forte dose s’est révélée rapidement efficace. L’hypothèse d’un neuro-Behçet a finalement été retenue.
Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5 ± 11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P = 0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities (n = 49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation (n = 25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta (n = 19), lower extremity arteries (n = 15), pulmonary arteries (n = 13), coronary arteries (n = 5), and subclavian arteries (n = 5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P = 0.024; 54.9 vs 76.5 %, P = 0.000; 19.6 vs 30.5 %, P = 0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P = 0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis.
Les manifestations ophtalmologiques au cours de la maladie de Behçet sont dominées par les uvéites, les vascularites rétiniennes et les thromboses veineuses rétiniennes. Nous rapportons une étude rétrospective portant sur tous les malades atteints de la maladie de Behçet, hospitalisés au sein du service d'Ophtalmologie de Fès de Janvier 2007 à Décembre 2009. Au total ce sont 33 patients qui sont inclut dans l'étudeLa moyenne d'âge est de 28,4 ans. Le délai moyen de consultation varie entre 1 jour et 3 ans. L'atteinte oculaire est bilatérale chez 26 patients (78,8% des yeux). L'AV est très basse avant le traitement: 28,8% à moins de 1/10. Les manifestations oculaires sont dominées par l'uvéite avec 77,3%, suivie de la vascularite rétinienne 54, 5% et la maculopathie 51, 5%. Le pourcentage des yeux dont l'AV était inférieur à 1/10 est passé à 19,7% après traitement. La fréquence de l'atteinte oculaire au cours de la maladie de Behçet est diversement appréciée selon les auteurs et selon le mode de recrutement des patients: elle va de 29% à 100%. L'atteinte uvéale est la plus fréquente des manifestations ophtalmologiques. Les lésions vasculaires rétiniennes sont dominées par la vascularite rétinienne essentiellement la périphlébite aussi bien au pôle postérieur qu'à la périphérie rétinienne. En l'absence de traitement et de mauvaise observance, le pronostic oculaire de la maladie de Behçet est très mauvais. La cécité s'installe dans 13 à 32%. Les manifestations ophtalmologiques au cours de la maladie de Behçet ont une valeur considérable, tant sur le plan diagnostique que pronostique. Notre étude a permis de décrire les aspects cliniques de l'atteinte oculaire de la maladie de Behçet dans un service d'Ophtalmologie tertiaire au Maroc.
Behçet's disease is a multisystemic vasculitis of unknown etiology, which is characterized by recurrent urogenital ulceration, cutaneous eruptions, ocular manifestations, arthritis and vasculitis, and its diagnosis is based on clinical criteria. Herein, we report a case of a patient with Behcet's disease, who was successfully managed with anticoagulant and anti-inflammatory therapy for disseminated venous thrombosis leading to pleural effusion, Budd-Chiari syndrome and central nervous system involvement following coronary artery bypass grafting surgery.
Behçet's disease (BD) is a multisystemic inflammatory disease characterized, on the one hand, by the frequency of benign mucocutaneous and articular manifestations, and on the other hand by the severity of ocular, neurological, vascular and digestive manifestations which may threaten functional or vital prognosis. The male BD predominance is obvious in many retrospective studies and a few studies only have focused on BD in women.
In this study, we report the epidemiological, clinical, outcome and therapeutic data of a series of 71 female BD compared to a series of 111 male BD. This retrospective study was conducted in the internal medicine department of Monastir university hospital over a 19-year period (1990 to 2008), including all patients followed-up and treated for BD.
The comparison of clinical manifestations of BD between men and women showed the predominance of erythema nodosum (P=0.016), necrotic pseudofolliculitis (P=0.001), and joint involvement (P=0.009) in women, while genital ulcers (P=0.039), ocular involvement (P=0.003) and vascular (P=0.002) involvement were more common in men.
Our results confirmed the results of previous studies regarding the benign nature of BD in women compared to men.
In Behcet's disease (BD), it is customary to believe that men are more affected than women, major organs are more involved in men, and they have worse outcomes. The male-to-female ratio is reported from 5.37 to 1 (Egypt), to 0.38 to 1 (US). If in the majority of reports BD was seen more frequently in men, in some others it was more frequent in women. The aim of this study was to examine a large cohort of patients, in whom manifestations were gender related, and to examine the strength of associations and their clinical relevance.
All patients of the BD registry, Rheumatology Research Center, Tehran University of Medical Sciences, entered the study (6702 patients). The percentage of 95 items was calculated in both genders (with their 95% confidence intervals), and were compared together by the chi-squared test. Odds ratio (OR) and relative risk (RR) were also calculated.
Forty-three out of 95 items were gender-related (29 for males, 14 for females) with a statistically significant difference by chi-squared. Significant OR (confidence interval not reaching 1) was found for 79 items. However, clinically significant OR (2 or more for men and 0.5 or less for women) showed an association only with 16 items; five with females and 11 with males. The most important was vascular involvement.
No strong association (OR of 2 or more) was found between the male gender and major organ involvement, except for vascular lesions.
Joint involvement in Behçet’s syndrome occurs in half of the patients in the form of arthritis or arthralgia. Behçet himself
described joint involvement in 1938, a year after the original description of the disease. It is usually mono or oligoarticular,
and can be symmetrical when not monoarticular. The arthritis usually lasts a few weeks and it seldom results in deformity
and radiological erosions. Chronic arthritis is seen only occasionally. The most frequently involved joints are the knees,
followed by ankles, wrists, elbows, and hands. Axial involvement in the form of spinal, shoulder, hip, and sacroiliac joints
is rare. In addition, back pain is quite rare and controlled studies have not shown an increased sacroiliac joint involvement.
Patients with Behçet’s syndrome and arthritis also have more acne lesions. Furthermore, patients with arthritis and acne lesions
have significantly higher enthesopathy scores than patients without acne. This suggests a possible pathogenetic link with
acne-associated arthritis. Laboratory features are generally nonspecific. The erythrocyte sedimentation rate (ESR) and CRP
may be mildly elevated but these may not correlate with the severity of arthritic attacks. Autoantibodies like rheumatoid
factor and antinuclear antibodies are absent. Synovial fluid is inflammatory with a predominance of polymorphonuclear leucocytes
although it has a good mucin clot. Synovial histology shows nonspecific inflammation. Fibromyalgia can be associated with
Behçet’s syndrome especially among the female patients. Generalized or localized myositis and osteonecrosis are infrequently
seen.
KeywordsArthralgia-Arthritis-Azathioprine-Behçet’s disease-Behçet’s syndrome-Colchicine-Fibromyalgia-Joint involvement-Myositis-Osteonecrosis-Synovial fluid-Synovial histology
Behcet’s syndrome (BS) is more prevalent along the ancient trading route known as the “Silk Road,” extending from the Mediterranean
to the countries in the Far East, where the prevalence of HLA-B5(51) is more frequent in the healthy population. This suggests
that the possible causative agent(s), including genetic factors such as HLA-B51, might have had spread along this route. Five
cross-sectional and population-based field studies showed that the prevalence of BS was between 20 and 421 among 100,000 adolescent/adult
population in Turkey. The lower two frequencies (20/105 and 80/105) come from Thrace, the European part. The disease is less common in Western Europe, USA, and in the rest of the world but
perhaps not as rare as it had previously been thought. A recent study showed that BS was as frequent (7.1/105) as other vasculitic diseases – 9/105 for the combination of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome
– in a suburb of Paris, France. We will review the epidemiological features of the field surveys of BS in Turkey and the rest
of the globe. We also look at the regional differences of some of the clinical and laboratory features. Gastrointestinal disease
is frequent in the patients from the Far East (especially Japan) while it is rather infrequent among those from Turkey. Moreover,
a high frequency of both pathergy test positivity and being a carrier of HLA-B5(51) are observed in patients from Japan and
the Mediterranean countries, in contrast to the patients from the United Kingdom and the USA.
KeywordsArthritis-Behçet’s disease-Behçet’s syndrome-Disease expression-Epidemiology-Erythema nodosum-Field survey-Folliculitis-Genital ulcer-HLA-B5(51)-Oral ulceration-Pathergy test-Regional difference-Uveitis
A number of inflammatory, infectious, neoplastic and idiopathic disorders affect the eye and the central nervous system (CNS) concurrently or at different time frames. These conditions pose a diagnostic challenge to the clinician since they may present with similar ocular and neurological manifestations. The purpose of this review is to describe major neurological syndromes including multiple sclerosis, Vogt-Koyanagi-Harada disease, other autoimmune syndromes, and several infectious diseases which may affect the eye. This article may serve as a guide for the diagnosis and treatment of such disorders. It should be noted that these conditions have been viewed from a neurologist's perspective thereby neurologic involvement is stressed.
Neurological involvement may be seen in 5-30% of the patients with Behcet's disease (BD). Occasionally, parenchymal neurological involvement in BD can present as a spinal cord syndrome. However, motor neuron disease-like presentation is extremely uncommon. Here we are reporting five patients (all male; median age, 38) fulfilling both International Study Group criteria for BD and El Escorial criteria for amyotrophic lateral sclerosis (ALS). These patients were identified by a questionnaire sent to the members of the Neuro-Behcet Study Group of the International Study Group for BD. Three out of five patients had only motor presentations. In two patients, sensory and urinary manifestations were present as well. Spinal cord MRIs were normal in all, and brain MRIs were normal in four patients; one patient had nonspecific white matter changes. Two patients passed away 1-3 years after diagnosis of ALS, and two patients were lost to follow-up 3 and 11 years after admission; one patient is still alive 3 years after onset. The patients that are presented here might represent a rare form of neurological involvement in BD as well as sole coincidence. Larger prospective series are needed to further answer this issue.
The risk that patients with Behçet's disease may develop various thrombotic complications has been previously described. Although vascular complications from Budd-Chiari syndrome associated with Behçet's disease have been described, the pathogenic mechanisms are still unknown. Severe vascular complications present in Budd-Chiari syndrome associated with Behçet's disease are very common among young male adults. The objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet's disease.
The risk that patients with Behçet's disease will develop thrombotic complications has been previously described. Although it is distributed worldwide, Behçet's disease is rare in the Americas and Europe. Even though the pathogenic mechanisms of vascular complications of Budd-Chiari syndrome in patients with Behçet's disease are unknown, severe vascular complications of Budd-Chiari syndrome associated with Behçet's disease seem to affect mainly young men.
We report a case of Budd-Chiari syndrome, a severe vascular complication that developed in a 25-year-old Afro-Brazilian woman with Behçet's disease.
Severe vascular complications of Budd-Chiari syndrome in patients with Behçet's disease are much more common in young adult male patients; we present a rare case of Budd-Chiari syndrome in a young Afro-Brazilian woman with Behçet's disease.
To identify the clinical picture of Behcet's disease in a large cohort of patients (6500) in Iran, over a period of 35 years, and compare them with other large series from around the world.
Patients with Behcet's disease from all over Iran were seen in the Behcet's Disease Research Unit by a multidisciplinary team (rheumatologists, dermatologists, and ophthalmologists). Diagnosis was based on 'expert opinion'. Data were collected on a standardized data sheet (105 items), and stored in an electronic database. Data were updated at each follow-up.
Male to female ratio was 1.22 : .00. The mean age at onset was 26 years ± 11.3. The frequency of symptoms were: oral aphthosis 97.3%, genital aphthosis 64.6%, skin manifestations 64.9% (pseudofolliculitis 54.5%, erythema nodosum 22.5%, other lesions 7%), pathergy phenomenon 52.5%, ophthalmologic manifes-tations 56.8% (anterior uveitis 41.2%, posterior uveitis 44.9%, retinal vasculitis 32.1%), joint manifestations 37.4% (arthralgia 17.2%, monoarticular arthritis 7.6%, oligoarthritis 16.8%, ankylosing spondylitis 2%), neurological manifestations 3.8% (central manifestations 3.5%, mononeuritis multiplex 0.3%), gastrointestinal manifestations 7.4%, vascular involvement 8.3% (phlebitis 5.7%, superficial phlebitis 2.2%, large vein thrombosis 1.1%, arterial thrombosis 0.154%, aneurysm 0.5%), epididymitis 4.7%, cardiac involvement 0.6%, and pulmonary involvement 0.9%. Sedimentation rate was normal in 46.5% of patients. Abnormal urine sediment was detected in 12.2%. HLA-B5 was present in 53.3% and HLA-B51 in 47.9% of patients.
Behcet's disease is mainly seen in young people. The most frequent symptoms are mucocutaneous, ocular and joint manifestations. Comparison with large series did not show major differences.
Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: “parenchymal” lesions, which include mainly meningoencephalitis as opposed to “extra-parenchymal” lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death.
To evaluate the prevalence of the clinical findings in Behçet's disease, we retrospectively analyzed the clinical data of 2147 Behçet patients from 9 to 87 years of age (mean age 38.3 years) followed up during the years 1976 through 1997. One thousand ninety three patients were male and 1054 patients were female. The male/female ratio was 1.03. The mean age at onset was 25.6 years. A family history of Behçet's disease was present in 7.3% of the patients. Positive pathergy was found in 1220 (56.8%) patients. All of the patients had mucocutaneous lesions. Out of the 2147 patients the disease manifested itself as only mucocutaneous involvement in 1168 patients. The prevalence of systemic manifestations was found as follows: 28.9% ocular involvement, 16.0% musculoskeletal involvement, 16.8% vascular involvement, 2.8% gastrointestinal involvement, 2.2% neurological involvement. Pulmonary involvement was seen in 20 (1.0%) patients, cardiac involvement was seen in 3 patients and renal involvement was observed in 2 patients. Male patients had vascular involvement 5.02, neurologic involvement 2.21 and ocular involvement 1.98 times more frequently than female patients.
Eye disease, arthritis, folliculitis, and thrombophlebitis were more common among males, and erythema nodosum among females, in 297 patients with Behćet syndrome (BS) at their first visit despite an identical disease duration. Younger males and females (age of onset 24 years or less) had a higher prevalence of eye disease and total clinical activity than did the older patients (age of onset 25 years or more). Among the 51 patients followed up for 52 months (SD 7 months) the total clinical activity became significantly less (p less than 0.05) in the whole group at the end of this period. This was particularly true for older females. While male sex and a younger age of onset are associated with more severe disease in BS, overall, the syndrome ran an improving or stable course over 4 1/2 years.
The clinical and laboratory profile of 58 consecutive patients satisfying the ISG 1990 criteria for the diagnosis of Behçet's
disease was analysed. It appears that Behçet's disease in India is predominantly ‘mucocutaneous’ and ‘arthritic’; ‘ocular’
and ‘neuro’ Behçet's being uncommon. In comparison to published literature, the onset of disease in this part of the world
is significantly delayed. The pathergy test is rarely positive. There is no significant difference in clinical presentation
and laboratory investigations between children and adults with this disease; also, no sex difference was observed. A combination
of oral steroids and colchicine gives good relief in most cases. Preliminary observations seem to reflect no definite association
of any known class I antigen to disease in this part of the world. A detailed study on immunogenetics is underway.
Anterior uveitis associated with Behcet's disease and ankylosing spondylitis preferentially occurs in adult men, which may suggest the effects of sex hormones on acute anterior uveitis. Recently, estrogen receptors in the vascular endothelium have been reported to be involved in several pathological conditions. In the present study, we examined the gender differences in susceptibility to endotoxin-induced uveitis (EIU) and the effects of estrogen on anterior inflammation. EIU was induced in adult male, female, and ovariectomized female Lewis rats (200 g) by hind footpad injection of 200 microg of LPS. In EIU, cellular infiltration was more marked in male than in female rats, and ovariectomy increased cellular infiltration. Treatment with 10 microg of 17beta-estradiol significantly reduced the cell number in male and ovariectomized female rats with EIU. Estrogen receptor immunoreactivity was found in the nucleus of vascular endothelium and in some stromal cells of the iris-ciliary body. Semiquantitative PCR revealed that E-selectin and IL-6 gene expressions were increased in rats following LPS injection, and an overdose of tamoxifen, an estrogen receptor antagonist, reversed the effect of 17beta-estradiol on E-selectin, but not its effect on IL-6. These observations suggested that the down-modulation of these inflammatory genes by estrogen may contribute to the reduction in cellular infiltration in acute anterior uveitis.
Introduction
Les manifestations cutanéomuqueuses sont fondamentales pour poser le diagnostic positif de la maladie de Behçet (MB). Ces manifestations peuvent précéder, accompagner ou suivre les autres atteintes systémiques au cette vascularite. Lorsqu’elles sont absentes, un diagnostic de certitude est alors impossible. Le but de notre travail est de décrire les différentes atteintes dermatologiques survenant au cours de la MB.
Patients et méthodes
Il s’agit d’une étude rétrospective portant sur 80 malades ayant la maladie de Behçet, hospitalisés au service de dermatologie du centre hospitalo-universitaire (CHU) Farhat Hached et au service de Médecine Interne du CHU Sahloul de Sousse sur une période de 17 ans (1997–2014).
Résultats
Il s’agissait de 57 hommes (71,25 %) et de 23 femmes (28,75 %). L’âge moyen était de 33 ans avec des extrêmes allant de 16 à 49 ans. L’aphtose buccale constituait la manifestation inaugurale de la MB dans 86,25 % des cas, l’érythème noueux dans 10 % des cas. Un syndrome de Sweet révélait la MB dans un cas. L’aphtose génitale était présente chez 32 patients, soit 40 % des cas, de localisation essentiellement scrotale chez les hommes et au niveau des grandes lèvres chez les femmes. Des aphtes au niveau de l’oropharynx et des amygdales étaient notés dans un cas. Les lésions de pseudo−folliculite étaient retrouvées chez 61 malades (76,25 %) avec une localisation plus fréquente au niveau du dos, du tronc et des cuisses. Le Pathergy test était positif chez 27 malades (33,74 %). Concernant les autres manifestations systémiques au cours de la MB, nous avons retrouvé des arthralgies inflammatoires chez 11 malades (13,7 % des cas), une atteinte oculaire chez 12 malades (15 %), une thrombose veineuse chez 20 malades (25 %) et une atteinte neurologique chez deux malades (2,5 %). Le traitement était basé sur la colchicine associée ou non à une corticothérapie et/ou à des immunosuppresseurs selon le degré et la gravité de l’atteinte systémique au cours de la MB.
Conclusion
Les manifestations cutanéomuqueuses sont diverses au cours de la MB. Elles sont nécessaires pour le diagnostic positif de cette vascularite. L’évolution de ces atteintes dermatologiques est très variable, caractérisée par des poussées et des rémissions durant quelques semaines, mois ou années.
Introduction
La maladie de Behçet (MB) est une maladie inflammatoire chronique systémique. L’atteinte oculaire est variable d’une région à une autre. Sa survenue aggrave le pronostic fonctionnel. On se propose de décrire les caractéristiques des patients suivis pour MB avec atteinte oculaire au centre de la Tunisie.
Patients et méthodes
Il s’agit d’une étude rétrospective colligeant les patients suivis pour MB entre les années 2000 et 2016. Les critères d’inclusion étaient ceux du groupe international d’étude sur la MB. Les patients ayant une atteinte oculaire étaient étudiés.
Résultats
Parmi les 243 patients suivis pour MB, l’atteinte oculaire était retrouvée dans 29,2 % des cas (n = 71). Le sex-ratio était de 3,43 et l’âge médian de 34 ans [15–55]. Les manifestations cliniques associées à l’atteinte oculaire étaient les aphtes buccaux (98,6 %), les aphtes génitaux (70,4 %), la pseudo-folliculite (70,4 %), l’atteinte neurologique (47,9 %), l’atteinte articulaire (45,1 %), un angio-Behçet (29,6 %) et un entéro-Behçet (9,9 %). La recherche du HLA B51 était positive dans 14,1 % des cas et négative dans 16,9 % des cas. L’atteinte oculaire se manifestait par une uvéite dans 84,4 %. Il s’agissait d’une uvéite antérieure, postérieure, intermédiaire et d’une panuvéite dans respectivement 33,8 %, 40,8 %, 2,8 % et 63,4 % des cas. Une vascularite rétinienne était notée dans 38 % des cas et une névrite optique dans 5,6 %. Les traitements prescrits étaient la colchicine (98,6 %), les corticoïdes (69 %) dont des Boli de Solumédrol dans 42,3 % des cas et les immunosuppresseurs (40,8 %). Les complications survenues au cours de l’évolution de la maladie étaient la cécité dans 28,2 % des cas, une cataracte dans 7 % des cas, un décollement de la rétine et une hypertonie du globe oculaire chacun dans 2,8 % des cas. Par ailleurs, l’évolution était favorable dans 25,4 % des cas.
Conclusion
La fréquence de l’atteinte oculaire dans la maladie de Behçet varie selon les séries allant de 28 à 50 % des cas. L’uvéite représente la principale atteinte oculaire de la maladie de Behçet. Elle fait partie des critères diagnostiques de la maladie. Le diagnostic de cette atteinte reste clinique. Son évolution se fait sur un mode chronique et peut compromettre le pronostic visuel. L’âge jeune, le sexe masculin et la sévérité de l’atteinte initiale constituent les principaux facteurs de mauvais pronostic visuel.
A statistical study was carried out on clinical symptoms of 2031 patients with Behcet's disease (complete type 928, incomplete type 1103). The records were compiled by sending individual inquiry sheets to about 7000 hospitals existing throughout Japan. Rate and the sex differences were studied concerning 4 major diagnostic criteria, i.e. recurrent aphthous ulceration on the oral mucous membrane, skin lesions, ocular symptoms and genital ulcers, and 5 minor criteria, i.e. arthritis, intestinal symptoms, epididymitis, vascular as well as neuropsychiatric symptoms and the prick test. Manifestation rates of the ocular symptoms, thrombophlebitis and skin hypersensitivity were higher in males than in females, but the reverse relation was found in the rates of erythema nodosum and genital ulcers. Clinical courses of the complete type were more unfavorable than those in incomplete type as was expected. Cases with the ocular symptoms showed high frequency of unfavorable clinical course indicating poor prognosis of the ocular lesions. Neuro Behcet syndrome was found in more than 10% of cases and was of poor prognosis. Among skin lesions, thrombophlebitis showed low association with major criteria, but positive prick test, found in about 80%, was highly associated with the major criteria, indicating its diagnostic significance.
A multicenter study of Behcet's disease in France confirms the male predominance (62 p. cent), the mean age of onset in the 4th decade, the frequent muco-cutaneous, ocular, articular manifestations (94 p. cent), the skin hypersensitivity (68 p. cent). The forms with arthralgia predominate; the knees are especially affected; arthritis has a favorable course; the para-articular forms are infrequent and in 4 cases, are associated with ankylozing spondylarthritis. HLA B5 is found in 47 p. cent of the cases. HLA B12 in 22 p. cent. HLA-B5 is associated with eye involvement in 62 p. cent of the cases, HLA-B12 is associated with skin involvement in 67 p. cent of the cases. The effectiveness of colchicin and, at a lesser degree, of steroids, is confirmed.
Uveitis is the most common ocular symptom in Behçet's disease. It is considered as a diagnostic criterion of the disease. Anterior uveitis is always non granulomatous, and sometimes associated with hypopion. Posterior involvement may include vitritis, retinal infiltrates, sheathing of retinal veins, occlusive vasculitis, and macular edema. Behçet's disease is chronic, and characterized by a spontaneously relapsing and remitting course. Male patients with younger age at onset and worse visual acuity at presentation, have higher risk of visual loss over time. The main goals in the management of patients with Behçet's disease-associated uveitis are rapid suppression of intraocular inflammation, preservation of vision, prevention of recurrences, and achievement of remission sustained after discontinuation of treatment. New therapeutic strategies such as interferon alpha and TNF alpha blockers have dramatically improved the visual prognosis of patients with intraocular inflammation related to this chronic and potentially blinding condition.
Introduction. - Behçet disease is a multisystemic vascularitis. Ocular affection is one of the major criteria of this disease. The aim of this study is to specify the clinical, therapeutical characteristics and the prognosis factors of the ocular affection in patients having BD and admitted to the dermatology department.Patients and methods. – It is a retrospective investigation carried out in the dermalogy department of Ibn Rochd university hospital center of Casablanca, Morroco, from Jannuary 1990 until December 2003. Two patient groups have been distinguished. The first one involved 50 patients (44,2%) having BD with ocular affection, and the second group involved 63 patients having BD without ocular affection.Results. – The mean age was 29±8 years in the first group VS 30±7,9 years in the 2nd group. The ocular affection was more frequent in males than in females (P
Purpose. – To describe epidemiological and clinical characteristics of neurological involvement in Behçet’s disease (BD) and to determine a subgroup of patients at high risk for this complication.Patients and methods. – The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behçet’s disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests.Results. – Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 ± 8.79 years. Nineteen patients (70.3%) had meningoparenchymal “MP” central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis:1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behçet “CAB” (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both “MP” and “CAB” involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in “CAB” subgroups than in subgroup 2.Conclusion. – Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behçet appears to be a protector factor against uveitis.
Purpose. – To define the epidemiology and clinical features of Behçet’s disease, giving special attention to unusual forms.Methods. – We retrospectively reviewed the medical records of 309 cases with joint manifestations among 450 cases of Behçet’s disease seen over a 20-year period who met the International Study Group of Behçet’s disease criteria.Results. – Joint manifestations were present in 68.3% and were inaugural in 34.5%. The knee and ankles were the joints most commonly affected. Monoarthritis, oligoarthritis and polyarthritis were seen respectively in 12, 13.5 and 19.8%. Sacroiliitis is observed in 6%. Unusual forms included destructive polyarthritis (two cases), popliteal cyst (two cases), myositis (two cases) and ankylosing spondylitis (two cases).Conclusion. – Joint manifestations are common in Behçet’s disease. They are frequently associated with erythema nodosum and necrotic pseudofolliculitis. Polyarthritis is not rare. Their unusual forms deserve to be known.
Purpose
Intrathoracic manifestations other then vascular aneurysm and thrombosis are rare in the course of Behçet's disease. Mediastinal fibrosis is also unusual.
Methods
Three cases of Behçet's disease with initial mediastinal fibrosis are reported. Diagnosis means, pathogenic mechanisms and therapeutic modalities are also more precisely defined.
Results
Three male patients (21, 25 and 27 years of age, respectively) were hospitalized for progressive vena cava superior syndrome accompanied by mediastinal fibrosis demonstrated by MRI and/or CT scan which both showed infiltration of the mediastinal fat. Multiple calcifications were also present in one patient. Histology was performed in one case. Thrombosis of the vena cava superior and innominata veins was associated with fibrosis in all three cases. Thrombosis of venae subclavia and left jugularis was also present in one case. Other manifestations leading to the diagnosis of Behçet's disease were demonstrated by the patient's history and, in one case, occurred on the fourth day of hospitalization. After initiation of a treatment including corticoids, anticoagulants and colchicine, the outcome was favorable in two cases. The third patient died.
Conclusion
Mediastinal fibrosis combined with Behçet's disease has rarely been described. However, it should be included in manifestations of the disease. The causative process which might be vasculitis has to be demonstrated.
La maladie de Behçet est une vascularite inflammatoire qui touche les artères et veines. L'atteinte vasculaire est une complication rare de cette maladie. Nous rapportons ici un cas de maladie de Behçet chez un patient qui a été hospitalisé plusieurs fois en raison des manifestations vasculaires.
Background: Behcet's Disease (BD) is a vasculitis of unknown origin. It is mainly seen around the Silk Road. The classification tree is the most sensitive and accurate criteria for the classification and diagnosis of the disease. The use of a classification tree necessitates having the diagram in hand. An arithmetic method was developed to replace the diagram by easy-to-remember figures.
Methods: Each criterion was given an appropriate value: ocular lesions were given 5 points, oral aphthosis 4 points, genital aphthosis 3 points, pathergy phenomenon 2 points, and skin manifestations 1 point. Seven points or higher values were necessary to classify a patient as having Behcet's disease. The arithmetic method was tested in 5276 BD patients and in 2407 control patients.
Results: The sensitivity of the classification tree was 97.2%, the specificity was 96.7%, and the accuracy was 97.1%. The arithmetic method gave exactly the same results.
Discussion: The arithmetic extrapolation of the classification tree is a new and simple way for the diagnosis and the classification of Behcet's disease.
This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD).
Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease.
We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified
for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients;
of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and
the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n=18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final
diagnoses were VST (12.2%), neuro-Behçet's syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet's syndrome
(0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached
a disability level of EDSS 6 or higher, and 95.7±2.1% of the patients were still alive. Having accompanying cerebellar symptoms
at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological
diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a
difference in pathogenesis.
Adamantiades-Behçet's disease is a universal disorder with varying prevalence, i.e. 80-370 patients per 100,000 inhabitants in Turkey, 2-30 patients per 100,000 inhabitants in the Asian continent and 0.1-7.5 patients per 100,000 inhabitants in Europe and the USA. Certain ethnic groups are mainly affected, while the prevalence of the disease seems to be strongly dependent on the geographic area of their residence. These data indicate environmental triggering of a genetically determined disorder. The disease usually occurs around the third decade of life, however, early and late onsets (first year of life to 72 years) have been reported. Juvenile onset disease rates from 7 to 44% in different ethnic groups; juvenile disease is less frequent, i.e. 2-21%. Both genders are equally affected. Familial occurrence has been reported in 1-18% of the patients, mostly of Turkish, Israeli and Korean origin, and is increased in patients with juvenile disease. Oral aphthous ulcers represent the onset sign in the majority of patients worldwide (47-86%). Oral aphthous ulcers (92-100%), genital ulcerations (57-93%), skin lesions (38-99%), ocular lesions (29-100%) and arthropathy (16-84%) are the most frequent clinical features; sterile pustules (28-66%) and erythema nodosum (15-78%) are the most common encountered skin lesions. The positivity of pathergy test varies widely in different populations (6-71%). HLA-B51 is associated with high relative risk for the disease in a small geographic area of the Mediterranean Sea countries and Southern Asia. Diagnosis can be established 2 to 15 years after the onset of the disease. Male gender, early development of the disease, and HLA-B51 positivity are markers of severe prognosis (mortality rates of 0-6%).
Among 250 patients with Behcet's disease, we describe 25 cases of angiographically proven cerebral venous thrombosis. Intracranial hypertension was the most frequent manifestation. Two initially untreated patients relapsed. Treatment of cerebral venous thrombosis consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others. Neurologic symptoms improved rapidly in all. Nineteen patients received long-term anticoagulation, and two received aspirin. Relapse of cerebral venous thrombosis or development of optic atrophy did not occur in treated patients. Partial or total recanalization of the occluded sinus was frequent. After more than 3 years of follow-up, the prognosis of dural sinus thrombosis is satisfactory.
The authors have reviewed the records of 316 patients with Behcet's disease diagnosed between 1981 and 1989 in the Internal Medicine Department of Casablanca's University Hospital, Morocco. Males aged between 20 and 40 years (78 percent) were most frequently affected (71 percent of the cases). All patients presented with muco-cutaneous lesions, including aphthous stomatitis (100 percent) and genital ulcers (85 percent). Skin hyperirritability was present in 77 percent. Ocular lesions were frequent (72 percent) and led to blindness in 88 patients. Articular manifestations occupied third rank in our series, with 60 percent of the cases. Three patients showed polyarthritis deformans, with destructive lesions in two cases and associated with Sjögren's syndrome in one case. Thrombophlebitis was present in 69 patients (superior vena cava thrombosis in 8 patients and Budd-Chiari syndrome in one), and it was complicated by pulmonary infarction in 6 cases. Other vascular lesions included middle cerebral artery thrombosis (1 case), cerebral arterial aneurysm (1 case) and pulmonary arterial aneurysm (1 case). Neurological manifestations were present in 50 patients (16 percent), in 13 of them related to mild intracranial hypertension. Ulcerative colitis was diagnosed in 2 cases and proteinuria in 4 others, with a nephrotic syndrome in 2 cases.
Vasculitis of arteries and veins is an important feature of Behçet's disease. When it occurs in systemic or pulmonary arteries or in large veins it is life threatening. Retinal vasculitis is the hallmark of eye disease, and much of the central nervous system disease is linked to small artery occlusions. Treatment with immunosuppressive drugs has improved the prognosis for sight and life.
A retrospective study of 177 patients (137 male, 40 female) with a disease that fulfilled the criteria of complete or incomplete Behçet's disease showed 106 thrombotic lesions of a venous trunk in 62 (35%) of these patients, superficial phlebitis and retinal vein thrombosis excluded. The incidence of thrombosis was unrelated to race and sex. The venous lesions were single in 38 cases and multiple in 24 cases, including 4 with more than 4 territories involved. Arterial lesions were also present in 7 cases. The lesions were located in the sural (n = 63), iliofemoral (n = 14), inferior vena cava (n = 13), superior vena cava (n = 3), axillary (n = 2) and cerebral (n = 13) veins or territories. Thrombosis developed during the first 4 years of Behçet's disease in one half of the patients. It preceded the aphthosis by 1 to 10 years in 11 cases, was concomitant with it in 15 cases and appeared more than 10 years after the diagnosis in 8 cases. Pulmonary embolism occurred in 11 patients, requiring clipping of the vena cava in 2, but it was never lethal. However, 2 of the 5 patients who died had venous thrombosis (Hughes Stovin syndrome). Thirty-eight patients could be followed up for 12 to 192 months (mean 43) under anticoagulant or antiplatelet therapy. Five vascular recurrences (13%) were observed, 2 of them (1 arterial graft thrombosis, 1 cerebral thrombosis) under anti-vitamin K agents. Cerebral thrombosis (9 men, 4 women) accounted for 27% of the neurological lesions; its prognosis was favourable in the 7 patients successfully treated with anticoagulants. Thrombosis could not be explained. Haemostasis studies showed a decrease of fibrinolytic activity compared to a group of normal subjects and an increase in fibrinogen, factor VIII and Willebrand's factor levels; these abnormalities did not seem to be specific. Thus, phlebitis truly is an element of Behçet's disease. Until its mechanism is better understood, effective anticoagulant therapy in cases with proven thrombosis and preventive antiplatelet therapy in the other cases seem to be rational measures.
The authors, in a series of 65 cases of Behçet's disease, notice 53% of rheumatological manifestations which are third in line after aphthosis and ocular disease. In order of decreasing frequency, it concerns arthritis (mono, oligo or poly-arthritis), arthralgias with an almost identical frequency, sacroiliitis, extraarticular involvement. These rheumatological manifestations are readily associated with necrotic pseudo-folliculitis and cutaneous aphthosis.
Sixty-five patients presenting with a vena cava syndrome have been examined over a 7 years period; incidence of systemic diseases as an etiologic factor has thus been studied. A systemic disease was diagnosed in 17 cases, that is 25 p. 100 of cases. This incidence is higher in inferior vena cava syndromes (15 cases out of 45). Two diseases appear as the main factors: on one hand, Behçet disease, liable for 8 vena cava thrombosis, 7 inferior and 8 superior thrombosis; on the other hand, severe disseminated erythematous lupus liable for 5 cases of inferior vena cava thrombosis. Other etiologies are mentioned such as: Churg and Strauss disease (1 case), Horton disease (1 case) and hemorrhagic rectocolitis (1 case). In the patients presenting with lupus, two died from various pulmonary embolisms; a vena cava clip was required in 3 cases. As for lupus, the immuno-hepatologic exploration did demonstrate a thrombopenia in 5 cases, an hemolytic anemia with a positive Coombs test in 2 cases, and a circulating anti-coagulant (antiprothrombinase type) in one case. Though high frequency of systemic diseases in this series may depend on the patients' recruitment, systemic lupus as well as Behçet diseases are to be researched in case of vena cava thrombosis, above all if diagnosed in a young patient and failing to be explained by a congenital abnormality of hemostasis.
To study the presentation of Behçet's disease (BD) in the Saudi population and to determine any differences in clinical manifestation and prognosis between men and women.
One hundred nineteen patients with BD attending the King Faisal Specialist Hospital and Research Centre (KFSH & RC) and the King Khalid University Hospital (KKUH) between 1979 and 1992 were studied.
The male to female ratio was 3.4:1. The mean age was 29.3; oral ulcer was present in all the patients (100%), genital ulcer 87%, ocular involvement 65%, skin manifestations 57%, and arthritis 37%. Central nervous system involvement was present in 52 patients (44%) of which 12 had benign intracranial hypertension (BICH), pleuropulmonary manifestation 16%, deep venous thrombosis 25%, arterial thrombosis and aneurysm 18%, gastrointestinal manifestations 4%, and epididymitis 4%. Significant proteinuria was present in 9 patients 7.5%, of whom 3 had renal biopsy. The pathergy test was positive in 15 of 85 (17.5%) patients. HLA-typing was performed for 85 patients and HLA B5(51) was positive in 61 (72%) patients compared with 26% general population.
Using the Fisher exact test, there was no significant difference in clinical manifestation or prognosis comparing men to women.
We analyzed the incidence, clinical features, and the long-term sequelae of arthropathy in 84 patients with Behçet's disease. After a mean disease duration of 44.7 months, 59 patients (70%) had at least 1 episode of arthropathy. Of these, arthropathy was present at the time of diagnosis in the majority of patients (75%). In the remainder, arthropathy developed 4 to 80 months (mean 29) after the diagnosis of Behçet's disease. Female patients were significantly more likely to have arthropathy than male patients (89 versus 56%, p = 0.006). The pattern of involvement was oligoarticular in 75%, monoarticular in 23%, and polyarticular in 2% of these patients. The most commonly involved site was the knee, followed by the ankle and the wrist. Radiologic examination of the involved site was normal in 80% of the patients. After a mean duration of arthropathy of 43.2 months, by the American College of Rheumatology (ACR) criteria, the functional class of the arthropathy was I-II in 88%, and III-IV in 12%. In conclusion arthropathy is a fairly common manifestation in patients with Behçet's disease, but crippling arthritis is relatively rare.
The Budd-Chiari syndrome is characterized by venous outflow obstruction of the liver, usually occurring as a consequence of thrombosis of the hepatic veins. Vasculitis is a major component of Behçet's syndrome. The aim of this study was to determine the incidence of hepatic vein thrombosis in patients with Behçet's disease and to estimate the effect of this entity upon the clinical features and course of Behçet's syndrome.
During an 8-yr period from 1985 to 1994, from a total of 493 patients with Behçet's disease seen at Hacettepe University Hospital, the incidence and effect of hepatic vein thrombosis on the clinical course of Behçet's syndrome was investigated. The hepatic vein thrombosis in each case was documented by hepatic venography and confirmed by digital subtraction angiography, computed tomography, ultrasonography, and liver biopsy. Coagulation parameters including protein C, protein S, and anti-thrombin III levels were easured in each case. The survival of cases with Behçet's syndrome complicated by Budd-Chiari syndrome and the effect of the Budd-Chiari syndrome on the survival of individuals with Behçet's syndrome were determined using the Kaplan-Meier technique.
Of the 493 cases of Behçet's syndrome, 53 (10.8%) were found to have one or more large vessel thrombosis. Of these 53 patients, 14 (26.4%) had hepatic vein thrombosis. Of these 14 patients, 8 had an additional inferior vena cava thrombosis and 4 had portal vein as well as total inferior vena cava thrombosis. Only two patients with isolated hepatic vein thrombosis were identified. These two patients and two additional patients with hepatic vein thrombosis plus thrombosis of the hepatic portion of the inferior vena cava are currently alive. Of the 10 patients with total inferior vena cava and hepatic vein thrombosis (4 also had portal vein thrombosis), all 10 died with a mean survival of 10.3 months. During the same time period, 37 patients obtained from a total of 1494 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction or inferior vena caval obstruction without Behçet's syndrome were found to have a Budd-Chiari syndrome. Of these 37 patients, 19 (51%) had an identifiable underlying disorder responsible for their hepatic vein thrombosis.
Based upon this experience, it appears as if Budd-Chiari syndrome is a relatively frequent complication of Behçet's disease. When individuals with Behçet's syndrome have BCS, concurrent thrombosis of the portal vein and inferior vena cava are often found, if the patency of these vessels is assessed. The clinical course of patients with Behçet's syndrome complicated by Budd-Chiari syndrome is poor. The extent of the vascular thrombosis within the inferior vena cava rather than the presence of the hepatic vein thrombosis per se is the major determinant of survival.
Vasculitis underlies most of the characteristic lesions of Behçet's disease and is obligatory for the diagnosis in populations at risk. Sporadic cases and similar presentations in non-Silk-Route patients should be labelled as Behçet's syndrome and carry different prognosis because of differences in underlying pathologic changes and pathogenesis. Ethnicity and vasculitis should be included in diagnostic criteria. Large vessel involvement is characteristic if less common.
Behçet's disease is a multisystem inflammatory disorder with unknown aetiology. It is a disease of young adults with a more severe course in males subjects. Its prevalence is high in the Mediterranean basin and Japan and has been linked with human leucocyte antigen B5 (HLA-B5) in those countries. According to the diagnostic criteria formed by the International Study Group, recurrent oral ulceration is a prerequisite, with two more typical symptoms or signs. Neurological involvement is one of the most devastating manifestations of Behçet's disease. The involvement is either caused by primary neural parenchymal lesions (neuro-Behçet) or secondary to major vascular involvement (vasculo Behçet). The course is relapsing-remitting, secondary progressive or primary progressive. The most commonly involved area is the brain stem, with additional symptoms or signs, hemispherical involvement with mental changes being the most common. Intracranial hypertension, usually owing to dural sinus thrombosis, has a special place in Behçet's disease. The most common clinical findings are pyramidal signs. Sensory symptoms or signs are much less frequent, and hemianopia and higher cortical function disturbances as well as pure cerebellar syndrome are rare features. Cerebrospinal fluid usually has a high protein content and/or pleocytosis. Notably, in the acute period most patients have lesions shown by magnetic resonance imaging (MRI) extending from the brain-stem to diencephalic structures. Differential diagnosis from multiple sclerosis can be difficult in patients with hemispheric white matter MRI hyperintensities. Immunosuppressives are used in treatment.
To gain additional knowledge on the joint manifestations of Behçet's disease, with special attention to unusual forms.
Retrospective review of 340 cases with joint manifestations identified among 601 cases of Behçet's disease seen over a 15-year period. All the patients met International Study Group for Behçet's disease criteria. Radiographs of all joints with arthritis were obtained. Starting ten years ago, a radiograph of the sacroiliac joint was taken routinely.
Joint manifestations were present in more than half the patients (56.57%) and were inaugural in 18.23% of cases. The knees and ankles were the joints most commonly affected. Monoarthritis and oligoarthritis were seen in 16.17% and 11.76% of cases, respectively and polyarthritis involving the large limb joints and the small joints of the hands and feet in 17.05% of cases. Unusual forms included polyarthritis with deformities and/or destruction (n: 8, including two patients who also met criteria for rheumatoid arthritis), pseudogout (n: 5), popliteal cyst (n: 3, including one case imitating deep vein thrombosis), myositis (n: 1), spondylarthropathy (n: 5), and Sjögren's syndrome (n: 2). Children were more likely than adults to have joint manifestations (73.68%) and polyarthritis (35.7%).
Joint manifestations are common in Behçet's disease. Their unusual forms deserve to be known since they can raise diagnostic problems when they are inaugural.
To evaluate the correlation between the age at onset of Behcet's disease (BD) and sex distribution, mode of disease appearance, and number of organs involved during the disease.
BD was defined according to the International Study Group criteria. Data from medical files and from patient interviews were collected. Results were analyzed for children and adults, according to age at disease onset. A systemic involvement index was calculated as the sum of visceral organ systems involved.
Fifty-nine patients with BD were studied, 26 male and 33 female. The mean age at disease onset was 8.4 +/- 4.5 years in children and 29.8 +/- 7.9 years in adults. The age of onset was significantly lower in male versus female patients. BD presented in children almost entirely as recurrent aphthous stomatitis, while in adults, less than one-third of patients presented first with oral ulcers. The mean age at disease onset of patients who presented first with oral ulcers was significantly lower than the age of patients presenting first with non-oral aphthosis. The mean systemic involvement index was higher in adult onset than in juvenile onset disease. A significant linear correlation was found between age of disease onset and total number of visceral organ systems involved.
BD was observed to occur earlier in males than in females. The first manifestation of BD in children is almost exclusively in the form of oral ulcers, while older patients have a large proportion of non-oral aphthosis as their first disease manifestation. Disease onset at an older age is positively correlated with increased disease spectrum.
