No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
News on Relapsing Polychondritis: The Patient's Experience
Arthritis Care & Research
Abstract
In this issue of Arthritis Care & Research, the authors of Patient‐Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis report for the first time the patient's view over the disease (1). Thanks go to them for this important step forward. The method used however, carries some limitations, as indicated very honestly by the authors.
This article is protected by copyright. All rights reserved.
... PFT (Pulmonary Function Test) done in our patient also showed severe obstructive lung disease. RPC is considered as an immune origin, involving humoral and cellular immunity as: there is an associated autoimmune disease in up to 30% of cases, chondritis lesions contain a CD4+ T-cell lymphocyte infiltrate and plasmocytes, as well as immune deposits, autoantibodies directed against type II collagen are detected in approximately 30% of cases, or against other types of minor collagen (IX and XI) or cartilage proteins, such as oligomeric proteins of the cartilaginous matrix (COMP) and matrilin-1, 4) a specific T-cell response against collagen II peptides, which represents 95% of cartilage collagen, or specific to matrilin-1 is sometimes observed and high-dose glucocorticoids (GCs) are generally effective [25,26,27]. Thus, according to current data, RPC is a probable autoimmune disease, with specific immunization against cartilage structures and matrillin-1 shown to be responsible for the tracheobronchial phenotype [26,27]. ...
... RPC is considered as an immune origin, involving humoral and cellular immunity as: there is an associated autoimmune disease in up to 30% of cases, chondritis lesions contain a CD4+ T-cell lymphocyte infiltrate and plasmocytes, as well as immune deposits, autoantibodies directed against type II collagen are detected in approximately 30% of cases, or against other types of minor collagen (IX and XI) or cartilage proteins, such as oligomeric proteins of the cartilaginous matrix (COMP) and matrilin-1, 4) a specific T-cell response against collagen II peptides, which represents 95% of cartilage collagen, or specific to matrilin-1 is sometimes observed and high-dose glucocorticoids (GCs) are generally effective [25,26,27]. Thus, according to current data, RPC is a probable autoimmune disease, with specific immunization against cartilage structures and matrillin-1 shown to be responsible for the tracheobronchial phenotype [26,27]. Nevertheless, with a multidisciplinary approach integrating his past histories, symptoms, and serologico-biochemical, bronchoscopic, radiographic, and spirometric findings, other diseases with similar clinico-radiological presentations such as tuberculous tracheitis, anti-neutrophilic cytoplasmic autoantibodies (ANCA)-associated vasculitis, sarcoidosis, amyloid deposition, tracheomalacia, excessive dynamic airway collapse, tracheal involvement in inflammatory bowel disease, rhinoscleroma, or tracheobronchopathia osteochondroplastica were ruled out. ...
Relapsing Polychondritis (RPC) is a rare systemic inflammatory disorder of unknown etiology and characterized by recurrent and progressive inflammation of the cartilaginous structures, particularly involving the auricles, nose and respiratory tract as well as extra-cartilaginous tissues, including eyes, heart, skin, central nervous and hematological systems. Its diagnosis can be difficult when the typical clinical features such as auricular chondritis are absent. Here, we report on a case of 43-year-old woman who presented with recurrent sore thorat, dysphagia, extertional dyspnea, cough and noisy breathing initially misdiagnosed as acute laryngitis who was eventually diagnosed as Relapsing polychondritis with tracheobronchial involvement. Chest computed tomography showed the diffuse involvement of tracheobronchial cartilage. Based on the, Damiani’s criteria, she was diagnosed as relapsing polychondritis even though there was no unique involvement of auricular cartilage, and high dose steroid and immunosuppressive therapy were then started. This case indicated that patients who have tracheobronchial cartilage involvement without definite auricular chondritis should be considered for relapsing polychondritis as a differential diagnosis. This case is reported to raise awareness of airway involvement in RPC and discuss its current management.
... Compared to the observations made in French and Japanese patients [28,29,34], our current study demonstrated a much stronger negative correlation between airway involvement and auricular chondritis (r = − 0.754, P < 0.001). Based on these findings, we divided RPC patients into 4 clinical patterns: Ear pattern (Subgroup A), Airway pattern (Subgroup B), Overlap pattern (Subgroup C) and Airway-Ear negative pattern (Subgroup D). ...
... The diagnostic criteria used in present study were listed in Table 4. Patients younger than 18 years or without complete electronic case files were excluded. Patients with positive anti-neutrophilic cytoplasmic antibody (ANCA) against proteinase-3 (PR3) were also excluded as suggested by Piette and colleagues [34]. The testing of ANCA was performed using Euroimmun AG detecting system (Lübeck, Germany), according to the procedures suggested by the instruction. ...
Background
Relapsing polychondritis (RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC.
Results
Sixty-six males and 60 females were included in this study. The average age at onset were 47.1 ± 13.8 years and the median follow-up period was 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r = − 0.75, P < 0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, a minority of limited RPC patients progressed into systemic form. Patients with Ear pattern had the highest survival rate and relatively lower inflammatory status.
Conclusions
RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement. The clinical patterns and presenting forms may evolve during follow-up. Our findings may facilitate early recognition of this rare disease.
... Compared to the observations made in French and Japanese patients [28,29,34], our current study demonstrated a much stronger negative correlation between airway involvement and auricular chondritis (r=-0.754, P<0.001). ...
... The diagnostic criteria used in present study were listed in table 4. Patients younger than 18 years or without complete electronic case les were excluded. Patients with positive anti-neutrophilic cytoplasmic antibody (ANCA) against proteinase-3 (PR3) were also excluded as suggested by Piette and colleagues [34]. The testing of ANCA was performed using Euroimmun AG detecting system(Lübeck, Germany), according to the procedures suggested by the instruction. ...
Background Relapsing polychondritis(RPC) is a rare autoimmune disease and its early diagnosis pose a challenge to medical committee. Defining the clinical patterns and disease course may help early recognition of RPC. Results Sixty-six males and 60 females were included in this study. Their average age at onset were 47.1±13.8 years with a median follow-up of 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r=-0.75，P<0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, some patients did become systemic form. Patients with Ear pattern had the highest survival rate and lower inflammatory status. Conclusions RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement, and clinical patterns and presenting forms can evolve during follow-up. Our findings may facilitate early recognition of this rare disease.
... Compared to the observations made in French and Japanese patients[28, 29,34], our current study demonstrated a much stronger negative correlation between airway involvement and auricular chondritis (r=-0.754, P<0.001). ...
... The diagnostic criteria used in present study were listed in table 4. Patients younger than 18 years or without complete electronic case les were excluded. Patients with positive anti-neutrophilic cytoplasmic antibody (ANCA) against proteinase-3 (PR3) were also excluded as suggested by Piette and colleagues [34]. The testing of ANCA was performed using Euroimmun AG detecting system(Lübeck, Germany), according to the procedures suggested by the instruction. ...
Background
Relapsing polychondritis(RPC) is a rare autoimmune disease, of which the diagnosis in early stage is challenging. Defining the clinical patterns and disease evolution may help early recognition of this rare disease.
Results
Sixty-six males and 60 females were included with onset age of 47.1±13.8 years and followed up for a median of 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r=-0.75，P<0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%) and patients with Ear pattern and Airway pattern were subdivided as limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern and some Airway-Ear negative pattern patients progressed into Ear pattern. Most of the limited RPC patients remained limited form during follow-up while some patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status.
Conclusions
RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement, and clinical patterns and presenting forms can evolve during follow-up. Our findings may facilitate early recognition of this rare disease and contribute to an updated classification criteria covering all the clinical spectrum of RPC
... Для подтверждения диагноза достаточно 1 большого и 2 малых критериев. В настоящее время продолжается разработка классификации и диагностических критериев РПХ [17,18]. ...
Relapsing polychondritis (RPC) is a rare disease, its diagnosis presents certain difficulties. This is due to the absence of characteristic clinical manifestations at the initial stages of the disease, late diagnosis and difficulties in selecting adequate therapy.The article presents a review of the literature on the diagnosis and treatment of RPC, as well as a clinical case with tracheobronchial tree and other organ systems involvement in the absence of classical auricular involvement.
Objective
The diagnosis of relapsing polychondritis (RP) is often mistaken or delayed. In this retrospective cohort, we aimed to unveil the causes responsible for such phenomenon, to determine the associated factors, and to compare diagnosis in clinical settings with the current diagnostic criteria.Method
Eighty-seven RP patients followed-up by rheumatologists from January 1, 2008, to October 31, 2018, were retrospectively analyzed.ResultsA total of 50 male and 37 female patients were included with a mean age of 45.9 ± 14.5 years. Ninety-three percent were initially admitted by non-rheumatologic specialists .Twenty-eight percent were correctly diagnosed, while 72% were misdiagnosed at the first visits, all by non-rheumatologic specialists. Patients admitted by non-rheumatologic specialists had increased odds of misdiagnosis (odds ratio [OR] = 1.3, 95% confidence interval [95% CI] 1.1–1.7, P = 0.000). Fifty-seven (65.5%) patients did not meet with Michet or Damiani criteria, with 16 (18.4%) patients diagnosed as partial RP and 41( 47.1%) patients diagnosed as limited RP.Conclusions
Incorrect and delayed diagnosis of RP is common in our cohort, and insufficient awareness of the disease in non-rheumatologic specialists at least partially contributes to this. It is imperative to revise the current criteria for early diagnosis.Key Points• Diagnosing relapsing polychondritis (RP) in early stage remains challenging after all these years, especially among non-rheumatologic specialists, indicating the importance of teaching non-rheumatologic specialists to improve their understanding of this rare disease.• Many RP patients did not fully meet with the current criteria, suggesting that revision of the current criteria is imperative for early diagnosis of this rare disease.
Recent studies show that relapsing polychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis and at each follow-up visit. Respiratory impairment is confirmed by computed tomography (CT) of the chest, including the cervical portion of the trachea, with end-inspiratory and dynamic expiratory scans, and pulmonary function tests. These investigations should be performed, even in asymptomatic patients, at the time of diagnosis, and repeated as necessary during follow-up. Bronchoscopy and a fortiori endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsing polychondritis has significantly improved the prognosis of patients, especially with the development of interventional fiberoptic bronchoscopy. However, relapsing polychondritis-related morbidity and mortality are still elevated, particularly in tracheobronchial disease.
We read with interest the letter from Shimizu et al. regarding their large survey of 121 Japanese physicians following a total of 239 patients with relapsing polychondritis (RP). Using correlation matrix, they found that airway involvement (laryngo and tracheobronchial (TB)) was strongly associated with nasal chondritis whereas the relationship was inverse with external ear chondritis suggesting that the nasal and the auricular chondritis define two subgroups of RP patients. This article is protected by copyright. All rights reserved.
This work is aimed to study the clinical and prognostic features of relapsing polychondritis (RP) in China. A total of 158 RP cases from 1985 to 2013 in China were included and compared with international case series in terms of clinical features, systemic involvement, differential diagnosis and prognosis. (1) The average age at the onset was 45.3 years old, the average age for initial symptoms was 14.4 months, female/male ratio was 0.7:1 and misdiagnosis rate was 47 %. (2) The incidence of arthritis was lower than that in Caucasians. The incidences of auricular chondritis (68 %: 84-95 %), ocular inflammation (44 %: 49-65 %) and renal involvement (3 %: 7-26 %) were lower, and laryngotracheal symptoms (69 %: 31-67 %), skin (46 %: 4-38 %) and neurological involvement (12 %: 2-8 %) were higher during the follow-up period. The proportion of associated autoimmune disease and systemic vasculitis were 5 and 3 %, respectively, similar to that in Japanese (4 and 2 %), but less than that in Caucasians (12-31 and 8-18 %) except the Francès's study (7 and 3 %). The primary death cause is respiratory failure due to RP, followed by lung infections and cardiovascular events. (3) Juvenile RP (onset ≤18 years) was more severe than adults, similar to results from the Caucasians. However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40-47 %), arthritis (100 %: 71-90 %), cardiovascular (14 %: 3-10 %) and skin involvement (20 %: 10-11 %) than Caucasian juvenile RP. Although sharing most of the clinical features with case series in previous literature, Chinese patients with RP have its unique characteristics.
Relapsing polychondritis is a rare disease characterized by cartilage inflammation. Our aim was to estimate the incidence, prevalence and mortality of relapsing polychondritis and describe the clinical features of relapsing polychondritis in a large population.
All participants diagnosed with relapsing polychondritis were sampled from the Clinical Practice Research Datalink. Prevalence and incidence rates for 1990-2012 were estimated. Relative mortality rates were estimated in a time-to-event framework using reference UK life tables. A questionnaire validation study assessed diagnostic accuracy.
There were 117 participants with relapsing polychondritis ever recorded. Fifty (82%) of 61 cases were validated by a physician and unconfirmed cases were excluded. The analysis included 106 participants (42 men, 64 women) diagnosed with relapsing polychondritis. The mean age (range) at diagnosis in men was 55 (range 17-81) years and in women 51 (range 11-79) years. The median interval from first symptom to diagnosis was 1.9 years. The incidence of relapsing polychondritis between 1990 and 2012 was 0.71 (95% CI 0.55, 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardized mortality ratio was 2.16 (95% CI 1.24, 3.51), P < 0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death.
The incidence of relapsing polychondritis may be lower than previously estimated, and diagnostic misclassification and delay are common. Mortality in relapsing polychondritis is more than twice that of the general population.
© The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.
Relapsing polychondritis (RP) is a multisystem disorder characterized by recurrent inflammation and destruction of cartilage. The aim of this study is to assess the clinical characteristics of patients with RP in Japan, which remain unclear.A survey was sent to 395 experienced clinicians who worked in Japanese major institutions. The questionnaire was designed to assess patients’ profiles, clinical features, diagnosis, treatments and present complications. The response rate was 30.6% and 239 RP patient data were collected. The average age of onset diagnosis was 52.7 years (range, 3-97) and the male-to-female ratio was 1.1:1. Clinical features of patients with RP in Japan were similar to previous studies. Airway and cardiac involvement, both of which were potentially serious complications of RP, were observed in 119 (49.8%) and 17 patients (7.1 %), respectively. Four patients (1.7%) had myelodysplasia. In addition to oral prednisolone (91.6%), patients received methotrexate (19.7%), cyclophosphamide (12.6%) and cyclosporine (8.4%) with clinical response rates of 64.0%, 66.7% and 73.7%, respectively. 42 patients (17.6%) required and underwent tracheotomy, including 12 patients (5.0%) who were treated with prednisolone only. 22 patients (9.2%) underwent stent placement and/or tracheotomy. The overall mortality rate was 9.0% (22 patients) and respiratory failure and pulmonary infection were the leading causes of death in patients with RP.Airway involvement of RP was fundamentally progressive and required frequent clinical checks and appropriate intervention with administration of both prednisolone and immunosuppressant. Cardiac involvement of RP was less common in Japan as compared with that in Western countries.
Indolent forms of mastocytosis account for more than 90% of all cases, but the types and type and severity of symptoms and their impact on the quality of life have not been well studied. We therefore performed a case-control cohort study to examine self-reported disability and impact of symptoms on the quality of life in patients with mastocytosis.
In 2004, 363 mastocytosis patients and 90 controls in France were asked to rate to their overall disability (OPA score) and the severity of 38 individual symptoms. The latter was used to calculate a composite score (AFIRMM score). Of the 363 respondents, 262 were part of an ongoing pathophysiological study so that the following data were available: World Health Organization classification, standard measures of physical and psychological disability, existence of the D816V KIT mutation, and serum tryptase level. The mean OPA and AFIRMM scores and the standard measures of disability indicated that most mastocytosis patients suffer from disabilities due to the disease. Surprisingly, the patient's measurable and perceived disabilities did not differ according to disease classification or presence or absence of the D816V KIT mutation or an elevated (> or = 20 ng/mL) serum tryptase level. Also, 32 of the 38 AFIRMM symptoms were more common in patients than controls, but there were not substantial differences according to disease classification, presence of the D816V mutation, or the serum tryptase level.
On the basis of these results and for the purposes of treatment, we propose that mastocytosis be first classified as aggressive or indolent and that indolent mastocytosis then be categorized according to the severity of patients' perceived symptoms and their impact on the quality of life. In addition, it appears that mastocytosis patients suffer from more symptoms and greater disability than previously thought, that mastocytosis may therefore be under-diagnosed, and that the symptoms of the indolent forms of mastocytosis might be due more to systemic release of mediators than mast cell burden.
Objectives
To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP).
Methods
We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months. Other outcomes were adverse drug reactions (ADRs), persistence of biologics and factors associated with a response.
Results
This study included 41 patients exposed to 105 biologics (tumour-necrosis factor (TNF) inhibitors, n=60; tocilizumab, n=17; anakinra, n=15; rituximab, n=7; abatacept, n=6). Overall response rate during the first 6 months of exposure was 62.9%. Complete response rate was 19.0%. Reduced corticosteroid doses were highly variable among patients. ADRs were mostly infections (n=42). Reasons for biologic withdrawal (73.3%) were insufficient efficacy (34.3%; ranging from 23.5% for tocilizumab to 72.7% for etanercept), loss of efficacy (18.1%) and ADRs (20.9%; mostly for anakinra: 46.7%). Persistence was comparable among biologic classes. Among TNF inhibitors, the highest persistence was observed with adalimumab. Differences in clinical response rates were observed depending on biologics and organ involvement. There were trends towards a lower response rate in cases with associated myelodysplastic syndrome and for a higher response rate for nasal/auricular chondritis, sternal chondritis and concomitant exposure to non-biologic disease-modifying antirheumatic drugs.
Conclusions
This study describes the efficacy of biologics for refractory RP. However, the number of complete responses was low and there were concerns about the risk of ADRs, particularly infections.
Objective:
To assess patient-reported symptoms and burden of disease in relapsing polychondritis (RP).
Methods:
Patients with RP completed a disease-specific online survey to identify symptoms attributed to illness. Patients were divided into subgroups based upon presence or absence of ear/nose, airway, or joint involvement. Pathway to diagnosis, treatment, and disease-related complications were assessed within each subgroup.
Results:
Data from 304 respondents were included in this analysis. Prior to diagnosis, most patients with RP went to the emergency room (54%), saw >3 physicians (54%), and had symptoms for >5 years (64%). A concomitant diagnosis of fibromyalgia and absence of ear/nose or joint involvement was associated with diagnostic delay >1 year. Common diagnoses prior to RP diagnosis included asthma in patients with airway involvement (35% vs 22%, p=0.03) and ear infection in patients with ear/nose involvement (51% vs 6%, p<0.01). Patients with joint involvement were more likely to receive a glucocorticoid-sparing agent (85% vs 13%, p<0.01). Most patients reported a major complication including disability (25%), tracheomalacia (16%) or hearing loss (34%). Patients with airway involvement reported more tracheomalacia (20% vs 4%, p<0.01). Disability (24% vs 7%, p<0.01) and hearing loss (39% vs 11%, p<0.01) were prevalent in the joint involvement subgroup.
Conclusion:
Patient-reported data in RP highlight a significant burden of disease. Patterns of organ involvement may lead to diagnostic delay and influence treatment decisions, ultimately impacting the development of disease-related complications. Timely diagnosis, standardization of treatment approaches, and prevention of disease-related complications are major unmet needs in RP. This article is protected by copyright. All rights reserved.
Relapsing polychondritis (RP) is a multisystem disorder of unknown etiology characterized by recurrent inflammation and destruction of cartilage tissues. We conducted a large-scale survey of 239 Japanese RP patients and reported that airway, cardiovascular, and central nervous system involvement was intimately associated with poor prognosis (1-4). Certain patients suffered from multiple organ involvement. To assess an association among each organ involvement, we conducted an exploratory study using correlation coefficients of 239 RP patients. This article is protected by copyright. All rights reserved.
The past 5 years have seen an increase in the use of a gluten-free diet outside a diagnosis of coeliac disease or IgE-mediated wheat allergy. This trend has led to the identification of a new clinical entity termed noncoeliac gluten sensitivity (NCGS). In this Review, we discuss the evidence for NCGS as demonstrated by the results of double-blind, placebo-controlled dietary rechallenge studies. Furthermore, the characteristic phenotype of individuals with NCGS is described as well as the symptom manifestations commonly reported after gluten exposure, which include intestinal symptoms consistent with IBS, and extraintestinal symptoms such as neurological dysfunction, psychological disturbances, fibromyalgia and skin rash. Moreover, emerging evidence suggests that NCGS can be associated with organic gastrointestinal pathologies, such as IBD, in which its presence might be a reflection of severe or stricturing disease. However, NCGS is not without its controversies and uncertainties, in particular pertaining to whether it is gluten or nongluten components of the grain evoking symptoms; evidence suggests that fermentable carbohydrates, amylase trypsin inhibitors and wheat-germ agglutinin can also be responsible culprits. Finally, we discuss the novel techniques that might help diagnose NCGS in the future.
Rheumatologists face unique challenges in discriminating between rheumatologic and non-rheumatologic disorders with similar manifestations, and in discriminating among rheumatologic disorders with shared features. The majority of rheumatic diseases are multisystem disorders with poorly understood etiology; they tend to be heterogeneous in their presentation, course, and outcome, and do not have a single clinical, laboratory, pathological, or radiological feature that could serve as a “gold standard” in support of diagnosis and/or classification. Thus, the development of criteria for use in routine clinical care and in clinical research has been an important focus in rheumatology. Improved understanding of disease pathogenesis and new diagnostic tools have led to reexamination of existing classification and diagnostic criteria with updated classification criteria for some diseases being endorsed recently (1, 2).
The American College of Rheumatology (ACR) Subcommittee on Classification and Response Criteria is responsible for guiding the development and validation of new classification and response criteria that are eventually considered for ACR endorsement. This includes review of proposals for the development of new criteria sets and providing the ACR leadership with recommendations for development and approval of new classification and response criteria sets (1, 3–5). The Subcommittee has previously published a guidance paper for the development of classification and response criteria (6). This prior work has provided details about the rationale for the ACR’s position on classification criteria, but clarification around the issue of diagnostic criteria was lacking. Indeed, the ACR endorsed preliminary diagnostic criteria for fibromyalgia (7) in 2010, which prompted discussions about whether the Subcommittee should also support the development and ACR endorsement of diagnostic criteria, in addition to that of classification and response criteria.
The primary objectives of this current article, by former and current members of the Subcommittee on Classification and Response Criteria, are to compare diagnostic and classification criteria, using specific examples from the published literature, and to clarify the ACR’s position on both types of criteria.
Background:
Women with inflammatory bowel diseases (IBD) commonly report an increase in their IBD symptoms related to their menstrual cycle. Hormonal contraceptives are safe for women with IBD and frequently used for reproductive planning, but data are lacking on their effect on IBD-related symptoms.
Methods:
We completed a cross-sectional phone survey of 129 women (31% response rate), aged 18 to 45 years, with IBD in an academic practice between March and November 2013. An electronic database query identified eligible women, and we sent an opt-out letter before contact. Questions included demographics, medical and reproductive history, and current/previous contraceptive use. Women were asked if/how their menses affected IBD-related symptoms and if/how their contraceptive affected symptoms. We calculated descriptive statistics and made comparisons by Crohn's disease versus ulcerative colitis on Stata V11.
Results:
Participants were predominately white (85%) and college educated (97%), with a mean age of 34.2 (SD 6.2, range 19-45) years. Sixty percent had Crohn's disease, and 30% had IBD-related surgery previously. Half of the participants were parous, and 57% desired future pregnancy. Of the participants, 88% reported current or past hormonal contraceptive use and 60% noted cyclical IBD symptoms. Symptomatic improvement in cyclical IBD symptoms was reported by 19% of estrogen-based contraceptive users and 47% of levonorgestrel intrauterine device users. Only 5% of all hormonal method users reported symptomatic worsening.
Conclusions:
In a subset of women with IBD, 20% of hormonal contraception users reported improved cyclical menstrual-related IBD symptoms. Health care providers should consider potential noncontraceptive benefits of hormonal contraception in women with cyclical IBD symptoms.
To define the natural history of relapsing polychondritis, the probability of survival and causes of death were determined in 112 patients seen at one institution. By using covariate analysis, early clinical manifestations were identified that predicted mortality. The 5- and 10-year probabilities of survival after diagnosis were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy. Only 10% of the deaths could be attributed to airway involvement by chondritis. Anemia at diagnosis was a marker for decreased survival in the entire group. There was an interaction between other disease variables and age in determining their impact on outcome. For patients less than 51 years old, saddle-nose deformity and systemic vasculitis were the worst prognostic signs. For older patients, only anemia predicted outcome. The need for corticosteroid therapy did not influence survival.
Dermatologic manifestations of relapsing polychondritis (RP) have been relatively poorly studied compared to other manifestations. In this study we describe dermatologic manifestations in a large series of patients with RP and the corresponding pathologic findings. In this retrospective, single-center review of 200 patients diagnosed with RP according to Michet's criteria, we analyzed separately those suffering from associated diseases with potential dermatologic involvement or chronic dermatitis. Skin or mucosal biopsies taken from 59 patients were examined without knowledge of the clinical data. Among the 200 patients with RP, 73 had chronic dermatitis or associated diseases with potential dermatologic involvement, especially hematologic disorders (n = 24) and connective tissue diseases (n = 22). Among the other 127 patients, 45 (35.4%) had dermatologic manifestations: aphthosis (n = 21; oral in 14 and complex in 7), nodules on the limbs (n = 19), purpura (n = 13), papules (n = 10), sterile pustules (n = 9), superficial phlebitis (n = 8), livedo reticularis (n = 7), ulcerations on the limbs (n = 6), and distal necrosis (n = 4). Dermatologic manifestations were the presenting feature of RP in 15 cases (12%), and appeared concomitantly (n = 23) or not (n = 22) with attacks of chondritis. Histologic findings included vasculitis (n = 19, leukocytoclastic in 17 and lymphocytic in 2), neutrophil infiltrates (n = 6), thrombosis of skin vessels (n = 4), septal panniculitis (n = 3), and minor changes (n = 2). Patients with and without dermatologic manifestations did not differ with regard to male/female ratio; age at RP onset; frequency of auricular, nasal, or tracheobronchial chondritis; or frequency of rheumatologic, ocular, audiovestibular, renal, arterial, or venous involvement. The frequency of dermatologic manifestations (91% versus 35.4%; p < 0.0001), sex ratio (18 male/4 female versus 44 male/83 female, p < 0.0001), and age at first chondritis (63.3 +/- 14 yr versus 41.4 +/- 17 yr; p < 0.0002) were significantly higher in the 22 patients with myelodysplastic syndrome than in the 127 patients without any associated disease. In conclusion, although dermatologic manifestations occur frequently in patients with RP, especially in association with myelodysplasia, they are nonspecific and sometimes resemble those observed in Behçet disease or inflammatory bowel diseases. Their presence in the elderly warrants repeated blood cell counts to detect a smouldering myelodysplasia.
Behçet's syndrome: a description of twenty-three cases
- Ja O'duffy Jd Levine
- Pseudo
An international consensus exercise to develop candidate items for classification criteria in relapsing polychondritis [abstract]
- Ferrada