-
[show abstract]
[hide abstract]
ABSTRACT: [(18)F]fluorodeoxyglucose-positron emission tomography (FDG-PET) has demonstrated its usefulness in giant cell arteritis, Takayasu's arteritis, and unclassified aortitis. FDG-PET imaging could be more effective than CT-scan or magnetic resonance imaging in detecting the earliest stages of vascular wall inflammation. Data are lacking regarding its accuracy for diagnosis and follow-up of vascular lesions in Behçet's disease. We report the case of a 32-year-old woman with history of Behçet's disease and fever of unknown origin revealing voluminous pulmonary arterial aneurysms. FDG-PET scan revealed a peripheral uptake of pulmonary aneurysms, underlying the inflammatory status of arterial aneurysms wall. Our patient was treated by glucocorticoids and intravenous cyclophosphamide and achieved remission of Behçet's disease. During follow-up, FDG-PET scan enabled to detect an early radiologic therapeutic response after 4 months, while CT angiography remained unchanged. We report the first evidence of usefulness of FDG-PET scan for therapeutic evaluation of arterial aneurysms in Behçet's disease. Further studies are warranted to determine the accuracy of FDG-PET scan in larger cohort of Behçet's disease patients with vascular involvement.
Joint, bone, spine: revue du rhumatisme 01/2013; · 2.25 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVES: To describe the small retinal and systemic vessel involvement in Takayasu's arteritis. METHODS: We described 3 patients with Takayasu's arteritis and small retinal vessel occlusion seen in our department between 2004 and 2011. We performed an extensive literature review and provided a global analysis of small retinal vessel involvement in Takayasu arteritis (i.e., total number of patients analyzed=9). RESULTS: Seven patients had small retinal artery occlusion, and two had venous involvement. Four cases were inaugural of the disease (44.4%). Takayasu's arteritis was extended (Type V) in the majority of patients presenting with small retinal vessel occlusion (5/9, 55.6%), and 8/9 reported cases (88.9%) presented with involvement of the supra-aortic branches. Immunosuppressive regimen allowed an improvement in 5/9 patients and stabilization in 1/9, but the situation worsened in 3/9 patients. The visual outcome was severe, and 3/9 patients (33.3%) experienced irreversible blindness. CONCLUSION: Occlusion of small retinal vessels is a rare and severe microcirculatory complication in Takayasu's arteritis, as well as necrotizing cutaneous vasculitis or myocarditis. Small retinal vessel involvement can be inaugural of the disease and seriously impact the visual prognosis in TA patients.
Autoimmunity reviews 06/2012; · 6.37 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population. CLINICAL DESCRIPTION: The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.
The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.
Diagnosis is only based on clinical criteria. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn's disease, Takayasu's arteritis, polychondritis or antiphospholipid syndrome need to be considered.
Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.
The prognosis is severe due to the ocular, neurological and arterial involvement.
Orphanet Journal of Rare Diseases 04/2012; 7:20. · 5.83 Impact Factor
-
Nicolas Noel,
Marie Hutié, Bertrand Wechsler,
Stéphane Vignes,
Du Le Thi Huong-Boutin,
Zahir Amoura,
Didier Dormont,
Véronique Delcey,
Marc Polivka,
Patrice Cacoub,
David Saadoun
[show abstract]
[hide abstract]
ABSTRACT: To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD).
We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD.
The median age was 39 (range 27-48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score ≥3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%) that led to complete clinical and imaging remission in 60.9% of patients. Two (8.7%) of the 23 patients with pseudotumoural NBD died of bedridden state complications.
The pseudotumoural form of NBD is a rare and life-threatening condition.
Rheumatology (Oxford, England) 02/2012; 51(7):1216-25. · 4.24 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by a foamy CD68+, CD1a- histiocyte tissue infiltration. Efficacy of standard doses of interferon-α-2a (IFNα) has been suggested in a small series but with variation, depending on the organs involved. Our aim was to report our single-center experience about the use of high-dose IFNα in ECD.
Twenty-four ECD patients have received high-dose IFNα (IFNα ≥18 mIU/wk or pegylated-IFNα ≥180 μg/wk). IFNα efficacy was evaluated clinically and morphologically using a standardized protocol (median follow-up 19 months).
Indication for treatment was central nervous system and/or heart involvement (n = 20), exophthalmos (n = 1), and standard-dose IFNα inefficacy (n = 3). High-dose IFNα was effective in 16 patients (67%) with improvement (n = 11, 46%) and stabilization (n = 5, 21%). Late and gradual improvement was observed during prolonged follow-up in most patients. The efficacy of high-dose IFNα was dependent on the organs involved: central nervous system and heart improvement or stabilization occurred in 7/11 (64%) and 11/14 (79%) patients, respectively. Six patients (25%) worsened. High doses of IFNα were well-tolerated: 13 (54.2%) patients had side effects but treatment interruption was infrequent (n = 3, 12.5%).
High-dose IFNα may be effective in severe ECD. Improvement may be slow, and high-dose IFNα treatment should be prolonged.
Seminars in arthritis and rheumatism 01/2012; 41(6):907-13. · 4.72 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.
Medicine 12/2011; 91(1):25-34. · 4.35 Impact Factor
-
David Saadoun,
Bouchra Asli, Bertrand Wechsler,
Habib Houman,
Guillaume Geri,
Kristel Desseaux,
Jean-Charles Piette,
Du Le Thi Huong,
Zahir Amoura,
Tara Ben Salem,
Philippe Cluzel,
Fabien Koskas,
Mathieu Resche-Rigon,
Patrice Cacoub
[show abstract]
[hide abstract]
ABSTRACT: The vasculitis of Behçet disease (BD) is distinctive because of involvement of both arteries and veins of all sizes. The concept of vasculo-Behçet disease has been adopted for cases in which vascular manifestations are present and often dominate the clinical features. While venous manifestations are frequent and have been reported in many publications, data regarding arterial lesions in patients with BD are rare and often isolated. In this study, we report the main characteristics, treatment, and long-term outcome of 101 patients with arterial lesions among a cohort of 820 (12.3%) BD patients. Factors that affect prognosis were assessed by multivariate analysis. There were 93 (91.2%) male patients; the median (Q1-Q3) age at diagnosis of BD was 33 (27-41) years. Arterial lesions included aneurysms (47.3%), occlusions (36.5%), stenosis (13.5%), and aortitis (2.7%). Lesions mainly involved the aorta (n = 25) and femoral (n = 23) and pulmonary (n = 21) arteries. Patients with arterial lesions were more frequently male (91.2% vs. 62.4%, respectively; p = 0.017) and had higher rates of venous involvement (80.4% vs. 29.8%, respectively; p < 0.001) compared to patients without arterial manifestations. Thirty-nine (38.6%) patients achieved complete remission. In multivariate analysis, the presence of venous involvement (odds ratio [OR], 0.29; 95% confidence interval [CI], 0.08-1.11) and arterial occlusive lesions (OR, 0.13; 95% CI, 0.01-1.25) were negatively associated with complete remission. The use of immunosuppressants (OR, 3.38; 95% CI, 0.87-13.23) was associated with the occurrence of complete remission. The 20-year survival rate was significantly lower in BD patients with arterial involvement than in those without arterial lesions (73% vs. 89%, respectively; p < 0.0001). In conclusion, the long-term outcome of arterial lesions in BD is poor, especially in the case of occlusive lesions and associated venous involvement. The use of immunosuppressants improved the prognosis.
Medicine 12/2011; 91(1):18-24. · 4.35 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Behçet's disease (BD) is a recurrent systemic inflammatory disorder of unknown origin characterized by oral and genital mucous ulcer, uveitis, and skin lesions. Involvement of large vessels, central nervous system (CNS), gastrointestinal tract and thrombotic events are less frequent but can be life threatening. The aim of this review is to provide new insights into the pathogenesis of BD. Over the past year substantial advances have been done in the understanding of the genetic [1,2] and immunology [3] of BD. BD is at the crossroad between autoimmune and autoinflammatory syndromes. In common with autoimmune diseases BD shares class I MHC association. However, in contrast to autoimmune disorders, BD has clinical features that seem to be mostly autoinflammatory. The pathogenesis of BD is still unknown, but major determinants of the genetic and immune system abnormalities have been reported recently. Triggering infectious factors are supposed to participate in the outbreak of BD in genetically predisposed patients. Two recent large genome-wide association study (GWAS) conducted in Turkey and Japan reported association between single nucleotide polymorphism (SNP) of interleukin (IL)-10 and IL-23R/IL-12RB2 genes and BD. New insights into the perturbations of T cell homeostasis of BD recently emerged. We have recently demonstrated the promotion of Th17 responses and the suppression of regulatory T cells (Tregs) that were driven by interleukin (IL)-21 production and that correlates with BD activity. Inflammatory cells within BD inflammatory lesions included mostly neutrophils, Th1 and Th17 cells, and cytotoxic CD8+ and γδ T cells. Altogether, the recent progresses in the knowledge of BD pathogenesis pave the way for innovative therapy.
Autoimmunity reviews 12/2011; 11(10):687-98. · 6.37 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: As impairment of diffusing capacity for carbon monoxide (DLCO) likely reflects underlying pulmonary vasculopathy in limited systemic sclerosis (lSSc), we examined whether DLCO could help to distinguish secondary from idiopathic Raynaud's phenomenon (iRP).
We compared pulmonary function test (PFT) results in 145 lSSc patients and 24 age- and sex-matched iRP patients. RP duration at time of PFT was similar in the two groups.
DLCO values were low (<80% of predicted) in 106 (73%) of the 145 lSSc patients, and in 69 (71%) of the 97 patients with early lSSc. Interstitial lung disease (ILD) was found in 10% of lSSc patients. DLCO was significantly lower in lSSc than in iRP (72±15% versus 89±9%, p<0.0001). When evaluated, alveolar capillary membrane conductance (Dm) was markedly lower in lSSc patients without ILD than in iRP patients (45±12% versus 71±2.5%, p=0.003), although capillary blood volume was not different. DLCO was low in 3 iRP patients (12.5%). The sensitivity and specificity of low DLCO values for early lSSc diagnosis in patients with Raynaud's phenomenon were 71% and 87.5%, respectively. Sensitivity was similar to that of anti-centromere-antibodies (75%) and nailfold capillary abnormalities (81%). A DLCO cutoff of <70% had a sensitivity and specificity of 41% and 100%, respectively. In multivariable analysis, age and low DLCO were the only independent predictors of death; the hazard ratio for DLCO ≤50% was 7.9 (95% CI 2.3-26, p=0.0007).
Isolated DLCO impairment is significantly more frequent in patients with lSSc than in patients with idiopathic iRP. DLCO measurement could be a useful diagnostic tool for lSSc.
European Journal of Internal Medicine 12/2011; 22(6):e80-6. · 2.00 Impact Factor
-
David Lebeaux,
Camille Francès,
Stéphane Barete, Bertrand Wechsler,
Odile Dubourg,
Jérôme Renoux,
Thierry Maisonobe,
Olivier Benveniste,
Marc Gatfossé,
Pierre Bourgeois,
Zahir Amoura,
Patrice Cacoub,
Jean-Charles Piette,
Damien Sène
[show abstract]
[hide abstract]
ABSTRACT: To analyse therapeutic management of eosinophilic fasciitis (EF).
We reviewed 34 adult patients with biopsy-proven EF. Analyses focused on the therapeutic management, including treatment modalities, responses and associated or predictive factors.
Thirty-four patients were included with a diagnosis age of 53 (15) years. They were featured by cutaneous manifestations (88%) including morphoea (41%), myalgia (86%) and hypereosinophilia (85%). Thirty-two patients (94%) were eligible for treatment evaluation and all received CSs as a first-line therapy. Fifteen patients (47%) received methylprednisolone pulses (MPPs) at treatment initiation and 14 patients (44%) received an immunosuppressive drug (ISD), usually MTX (86%), as a second-line therapy. Complete remission was achieved for 69% of patients, remission with disability 19% and failure 12%. A poor outcome was associated with a diagnosis time delay of >6 months [odds ratio (OR) = 14.7] and the lack of MPPs (OR = 12.9).
Our study reports new insights into the therapeutic management of EF: (i) CS treatment remains the standard therapy for EF, taken alone or in association with an ISD; (ii) MPPs at initiation of treatment are associated with a better outcome and a lower need of ISD use; (iii) an ISD, usually MTX, might be useful as a second-line therapy, mainly in patients with morphoea-like lesions. Naturally, these practical conclusions should be confirmed by a prospective and multicentre study.
Rheumatology (Oxford, England) 11/2011; 51(3):557-61. · 4.24 Impact Factor
-
Peggy Reiner,
Damien Galanaud,
Gaëlle Leroux,
Marie Vidailhet,
Julien Haroche,
Du Le Thi Huong,
Camille Francès,
Thomas Papo,
Christian de Gennes,
Lucile Musset, Bertrand Wechsler,
Zahir Amoura,
Jean-Charles Piette,
Nathalie Costedoat-Chalumeau
[show abstract]
[hide abstract]
ABSTRACT: The aim of this work was to describe chorea during systemic lupus erythematosus or antiphospholipid antibodies and its long-term outcome.
We retrospectively analyzed clinical features, laboratory findings, imaging characteristics, and outcome in a series of 32 patients.
Most patients were women (28 of 32), and mean age at onset of chorea was 20.6 (9-62) years. Chorea was inaugural for 28 patients. Improvement was observed with various treatments. During follow-up (12.2 ± 11.3 years), severe manifestations of systemic lupus erythematosus were rare. Antiphospholipid antibodies were repeatedly positive for 90% of the patients. Twelve patients developed arterial thrombosis. Prophylactic treatment with antithrombotic therapy might reduce the risk of further thrombosis (8% versus 57%; P = 0.01). Cardiac valvulopathy occurred in 22 patients during follow-up. Chorea relapsed in 8 cases.
Chorea had a good outcome in itself. This long-term follow-up shows, for the first time, that these patients have substantial risk for further arterial thrombosis.
Movement Disorders 07/2011; 26(13):2422-7. · 4.51 Impact Factor
-
Guillaume Geri,
Benjamin Terrier,
Michelle Rosenzwajg, Bertrand Wechsler,
Maxime Touzot,
Danielle Seilhean,
Tu-Anh Tran,
Bahram Bodaghi,
Lucile Musset,
Vassili Soumelis,
David Klatzmann,
Patrice Cacoub,
David Saadoun
[show abstract]
[hide abstract]
ABSTRACT: Behçet disease (BD) is a chronic systemic inflammatory disorder of unknown etiology.
To determine the nature of T cells driving inflammatory lesions in BD.
T cell homeostasis and cytokines production were analyzed in peripheral blood and brain inflammatory lesions from 45 adult patients with BD (active and untreated BD [n = 25] and patients in remission [n = 20]) and 20 healthy donors, using Luminex, flow cytometry, immunohistochemistry, and immunofluorescence analysis.
We found a marked increase in T(H)17 cells and a decrease in the frequency of CD4(+) forkhead box P3(+) regulatory T cells (Tregs) in peripheral blood that were induced by IL-21 production and that correlate with BD activity. The addition of serum from patients with active BD in a sorted CD4(+) T cells culture of healthy donors induced a significant and dose-dependent production of IL-17A and a decrease in forkhead box P3 expression. We demonstrated the presence of IL-21- and IL-17A-producing T cells within the cerebrospinal fluid, brain parenchyma inflammatory infiltrates, and intracerebral blood vessels from patients with active BD and central nervous system involvement. The stimulation of CD4(+) T cells with IL-21 increased T(H)17 and T(H)1 differentiation and decreased the frequency of Treg cells. Conversely, IL-21 blockade with an IL-21R-Fc restored the T(H)17 and Treg homeostasis in patients with BD.
We provided here the first evidence of the critical role of IL-21 in driving inflammatory lesions in BD by promoting T(H)17 effectors and suppressing Treg cells. IL-21 represents a promising target for novel therapy in BD.
The Journal of allergy and clinical immunology 07/2011; 128(3):655-64. · 9.17 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Systemic lupus erythematosus (SLE) affects women of child-bearing age. Combined oral contraceptives can worsen the course and increase the risk of thrombosis. The objectives of this study were to provide an alternative contraception and thus evaluate the gynecological tolerability of pregnane progestins (PPs) in SLE patients. Systemic lupus erythematosus disease activity and vascular tolerance were also reported.
We used two PP with antigonadotropic potencies, chlormadinone acetate (CMA, 10 mg/day) and cyproterone acetate (CPA, 50 mg/day), administered orally for contraception in 187 SLE patients observed for 46±34.6 months (mean±S.E.), i.e., 6854 women-months.
The gynecological tolerability was satisfactory: breakthrough bleeding was reported in 17.7% patients using CPA and 12.6% patients using CMA. No pregnancy was observed in the women followed in this cohort study. One deep vein thrombosis, one myocardial infarction, and one tibial posterior arterial occlusion were observed, giving an incidence for venous thromboembolism of 1.39/year×1000 women (95% CI 0-4.12) and for macroarterial disease an incidence of 2.79/year×1000 women (95% CI 0-6.65). Disease activity was less than before progestins.
Pregnane progestin contraception is effective and well tolerated, thus providing SLE patients an excellent contraceptive alternative to the currently used methods.
Contraception 03/2011; 83(3):229-37. · 2.72 Impact Factor
-
Laurent Arnaud,
Baptiste Hervier,
Antoine Néel,
Mohamed A Hamidou,
Jean-Emmanuel Kahn, Bertrand Wechsler,
Gemma Pérez-Pastor,
Bjørn Blomberg,
Jean-Gabriel Fuzibet,
François Dubourguet, [......],
Juan Salvatierra,
Carlos Graux,
Patrice Cacoub,
Véronique Delcey,
Claudia Dechant,
Pascal Bindi,
Christiane Herbaut,
Giorgio Graziani,
Zahir Amoura,
Julien Haroche
[show abstract]
[hide abstract]
ABSTRACT: Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would influence survival in a large cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical presentation of 53 patients with biopsy-proven ECD, and we performed a survival analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 women) with biopsy-proven ECD were followed up between November 1981 and November 2010. Forty-six patients (87%) received interferon-α and/or PEGylated interferon-α. Multivariate survival analysis using Cox proportional hazard model revealed that central nervous system involvement was an independent predictor of death (hazard ratio = 2.51; 95% confidence interval, 1.28-5.52; P = .006) in our cohort. Conversely, treatment with interferon-α was identified as an independent predictor of survival (hazard ratio = 0.32; 95% confidence interval, 0.14-0.70; P = .006). Although definitive confirmation would require a randomized controlled trial, these results suggest that interferon-α improves survival in ECD patients. This may be seen as a significant advance, as it is the first time a treatment is shown to improve survival in this multisystemic disease with high mortality.
Blood 01/2011; 117(10):2778-82. · 9.90 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To study the pediatric presentation and evolution of relapsing polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage.
We retrospectively collected data from 10 patients observed in 3 French hospitals for relapsing polychondritis, with an age at onset <18 years. We also analyzed 37 cases of pediatric-onset RP from a systematic review.
The mean age at first symptoms was 8.6 years, and the sex ratio was 6 male patients and 4 female patients. Children came to medical attention with joint pain, ocular inflammation, and chondritis. Outcomes included severe visual impairment, chronic destructive chondritis, and 1 death caused by aortic dilatation. Treatment mainly consisted of non-steroidal-anti-inflammatory drugs, corticosteroids, and immunosuppressants. Growth was normal in 7 examined patients. Systematic literature review also suggested a high number of tracheostomy in pediatric cases, but this was not confirmed in our series.
RP in childhood shares the main clinical features of its adult counterpart, including destructive chondritis and systemic symptoms, but unlike adults, children frequently have a family history of autoimmunity and infrequently have other associated autoimmune diseases. RP can be fatal; close screening for complications is mandatory. Growth does not appear to be impaired by cartilage inflammation.
The Journal of pediatrics 10/2009; 156(3):484-9. · 4.02 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a).
Retrospective study.
Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppressive agents were included. IFN-alpha2a was administered subcutaneously (three million units thrice a week). Ophthalmologic examination and fluorescein angiography associated with laboratory tests were performed at regular intervals. Main outcome measures were visual acuity and recurrence of uveitis attacks before, during, and after initiation of IFN-alpha2a therapy.
Control of inflammation was achieved in 28 of 32 patients (88%). Mean observation period of the 28 responder patients was 70.6 months (range, 30.3 to 129.2 months). Median visual acuity improved from 0.52 to 0.33 (logarithm of the minimum angle of resolution units; P = .005) two years after initiation of IFN-alpha2a therapy. The relapse rate decreased significantly during IFN-alpha2a treatment from 1.68 +/- 1.22 relapses/patient/year to 0.11 +/- 0.20 relapses/patient/year (P < .0001). IFN-alpha2a was discontinued in 19 of 28 patients (68%) after 32 months of treatment (range, 16 to 50 months). Mean follow-up after IFN-alpha2a discontinuation was 43 months (range, 11 to 84 months). After IFN-alpha2a discontinuation, the relapse rate increased from 0.08 +/- 0.21 relapse/person/year to 0.74 +/- 1.40 relapse/person/year (P = 0.04).
IFN-alpha2a is efficient and safe for the long-term management of severe uveitis associated with Behçet disease. Meanwhile it seems to be a suspensive therapeutic strategy, even though long-term remission is possible in some patients.
American journal of ophthalmology 01/2009; 146(6):837-44.e1. · 3.83 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To estimate the prevalence of Behçet's disease (BD) in a multiethnic population living in France, with particular focus on disease risk among immigrants.
The study was conducted in a county in the Paris metropolitan area that is home to 1,094,412 adults (ages > or =15 years), of whom 26% are of non-European ancestry. Patients with BD living in this area during 2003 were identified using 3 sources (hospitals, community physicians, and the National Health Insurance database), and diagnoses were verified using the International Study Group criteria. Standardized, year-2003 prevalence rates were computed for the overall population and for each ethnic group. Stratified prevalence rates according to age at immigration to France were calculated to investigate the relationship between age at immigration and BD risk.
Seventy-nine subjects fulfilled our search criteria. The overall prevalence per 100,000 adults was 7.1 (95% confidence interval [95% CI] 3.5-14.4), and the prevalence for populations of European, North African, and Asian ancestry was 2.4 (95% CI 0.6-7.2), 34.6 (95% CI 24.4-47.5), and 17.5 (95% CI 10.7-27.2), respectively. Within the migrant population of either North African or Asian ancestry, BD prevalences were similar for residents born in France, residents <15 years old at immigration, and residents > or =15 years old at immigration.
Our findings indicate that the prevalence of BD among immigrants of North African or Asian ancestry is significantly higher than that in the European-origin population, and comparable with rates reported from North Africa and Asia. Moreover, our results suggest that BD risk is not related to age at immigration. These findings support the hypothesis that BD has a primarily hereditary basis.
Arthritis & Rheumatism 12/2008; 58(12):3951-9. · 7.87 Impact Factor
-
Blood 07/2008; 111(11):5413-5. · 9.90 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Arterial aneurysms are characteristic of medium-size vessel vasculitis but are a very unusual feature of Wegener's granulomatosis (WG). We describe a typical WG case, complicated by arterial aneurysms and review previously reported cases.
Medline database search of cases published between January 1978 and July 2006, in English, reporting arterial aneurysms complicating WG.
Five years after diagnosis, a 29-year-old man with typical WG developed macro- and microaneurysms located on branches of the hepatic and renal arteries during a disease relapse. The main symptoms were abdominal pain, vomiting, and altered general status. He was successfully treated by coil embolization in combination with prednisone, intravenous mycophenolate mofetil, and high-dose immunoglobulins. Twelve additional cases of WG complicated by arterial aneurysms are reported in the English literature. This represents a life-threatening complication since rupture occurred in half of the patients.
Although small-vessel injury predominates in WG, inflammation of medium-size arteries may occur and lead to aneurysm formation. Abdominal angiography should be recommended when unexplained abdominal pain occurs during a WG flare.
Seminars in Arthritis and Rheumatism 03/2008; 37(4):265-8. · 4.97 Impact Factor
-
Olivier Fain,
Mohamed Hamidou,
Patrice Cacoub,
Bertrand Godeau, Bertrand Wechsler,
Jacques Pariès,
Jérôme Stirnemann,
Anne-Sophie Morin,
Marc Gatfosse,
Thomas Hanslik, [......],
Anne-Marie Piette,
Jean-Charles Piette,
Thierry Ponge,
Xavier Puechal,
Jérôme Rossert,
Françoise Sarrot-Reynauld,
Didier Sicard,
Jean-Marc Ziza,
Marcel-Francis Kahn,
Loïc Guillevin
[show abstract]
[hide abstract]
ABSTRACT: To describe characteristics and outcomes of vasculitides associated with malignancies.
The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy.
Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02).
Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.
Arthritis & Rheumatism 01/2008; 57(8):1473-80. · 7.87 Impact Factor
