International Criteria For Behcet's Disease Research Articles

Update on Behçet syndrome

Behçet syndrome (BS) is avasculitis of variable vessels with multiple organ manifestations. This article gives an overview of innovations in the last 2 years. A literature search was carried out using the keyword "Behcet" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance. With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures. It is now classified as MHC-I-opathy, i.e., adisease that has astrong association with HLA classI antigens, which also play aprominent role in the pathogenesis. The diagnostic international criteria for Behcet's disease (ICBD) from 2014 with ascore of 4 points or more that makes the diagnosis of BS probable have become established; however, in countries with alow prevalence of BS, the differential diagnosis of BS from other diseases is difficult and ahigher point limit in the diagnostic score seems to make sense in order to avoid incorrect diagnoses. Clusters or phenotypes of the disease have now been described in various countries in which different symptom complexes frequently occur together; however, the clusters differ between the different countries of origin and depending on the age of the patients. Sonography of the common femoral vein with specific wall thickening in BS patients has been established as an additional tool for the differential diagnosis. Typical characteristics of oral aphthae in BS were also described and the frequency of positivity in the pathergy test could be significantly increased using pneumococcal antigens as the reagent. The treatment recommendations of the EULAR from 2018 still apply; in treatment-refractory cases, tocilizumab, secukinumab, Janus kinase inhibitors (JAKi) and ustekinumab have now also been successfully used. The new EULAR treatment recommendations are expected in 2025.

Drug Retention Time of Immunosuppressive Therapy in Behcet’s Uveitis

ABSTRACT Purpose Behcet’s Disease is a chronic multisystem vasculitis associated with a blinding uveitis. Few comparative studies exist between conventional disease-modifying antirheumatic drugs (DMARDs) and biologic DMARDs in Behcet’s uveitis (BU). We therefore used drug retention time (DRT), an accepted surrogate measure of pharmacological efficacy and tolerability, to compare these treatments in patients with BU. Methods Retrospective chart review of patients who met the revised International Criteria for Behcet’s Disease (ICBD) treated at the Royal Victorian Eye and Ear Hospital, Australia, between 1985–2021. DRT was analysed with Kaplan-Meier plots and defined as total time on drug in the first medication-period for each DMARD in each patient. Results Forty-eight patients (37 males) with median age of 28.6 years were followed-up for a median of 8.0 years. At initial presentation, half had bilateral disease and median logMAR visual acuity was 0.176 (Snellen 6/9) in 62 uveitic eyes (16 anterior uveitis, 11 intermediate, 2 posterior, and 33 panuveitis). Thirty-three patients met ICBD initially. Prescribed corticosteroid-sparing agents were Cyclosporin (N = 24), Mycophenolate (N = 22), Azathioprine (N = 22), Methotrexate (N = 16), and Adalimumab (N = 15). Median DRT was 14.0, 27.4, 8.3, 24.0, and 52.0 months, respectively. DMARDs were discontinued 116 times and adverse effects (N = 37) were the main reason for cessation. Over time, patients were switched from Cyclosporin to Adalimumab earlier in the disease course due to poorer tolerance of adverse events. Conclusion Adalimumab’s drug retention time was found to be similar to and possibly better than cDMARDs in patients with BU, who often suffer from vision-threatening disease at first presentation.

Effect of Behcet's disease-associated oral ulcers on oral health related quality of life.

The current study assessed the clinical characteristics of patients suffering from Behcet's disease (BD)-associated oral ulcers and investigated the effect of these oral ulcers on the oral health-related quality of life (OHRQoL). This study included BD patients suffering from active oral ulcers, defined by the revised International Criteria for BD (ICBD) criteria. We collected BD and oral ulcers characteristics. The oral ulcer activity index and OHRQoL were measured by composite index (CI) and Oral Health Impact Profile-14 (OHIP-14), respectively. Genital and ocular manifestations were the most frequent. Ocular manifestations were the main diagnostic manifestation. The mean age of BD diagnosis and first oral ulcer appearance were 32.15±8.96 and 29.62±9.04, respectively. Minor oral ulcers were more frequent; solitary or multiple. CI revealed that pain was more severe than functional disability. OHIP-14 showed that patients suffered more often from pain and difficulties in eating. Patients reported feeling tense, being irritable, doing usual job with difficulty, and having less satisfying life. BD-associated oral ulcers lead to poor quality of life. Female gender, multiple ulcers, and buccal mucosa were associated with more severe pain and functional disability as well as poorer quality of life.

Pattern and Visual Prognostic Factors of Behcet's Uveitis in Northwest Iran.

PurposeTo investigate the pattern of ocular involvement in Behcet's disease (BD) with predictors of patients' final state of vision.MethodsThis historical cohort encompassed the clinical records of 200 patients diagnosed according to the International Criteria for BD (ICBD), over a period of 17 years between 2004 and 2021.ResultsThe prevalence of Behcet's uveitis (BU) was more common in females and patients in the fourth decade of life. Ninety-five patients (47.5%) had evidence of ocular involvement in the initial ophthalmologic evaluation, and 171 patients (85.5%) manifested evidence of BU during the follow-up visits of which bilateral non-granulomatous panuveitis was the most common anatomical pattern of involvement (32.9%) followed by posterior (27.6%), anterior (26.5%), and intermediate (13.8%) uveitis. The prevalent accompanying signs were oral aphthous (67%), skin lesions (29%), and genital ulcers (19.5%). Cystoid macular edema (CME) was the most frequent ocular complication (62%), followed by cataract (57.5%) and epiretinal membranes (ERM) (36.5%). Univariate analysis showed the following determinants: male gender, younger age at onset, panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis as poorer visual prognostic factors of the disease. Multivariate analysis demonstrated a higher chance of poor visual prognosis of BD in patients with panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis.ConclusionThis cohort study demonstrated an overview on epidemiological patterns of BU along with the visual prognostic factors in Iranian patients.

P102 The hidden face of Behcet’s syndrome; central nervous system involvement, a single-center experience

Abstract Background/Aims Neurologic involvement in Behcet’s syndrome (BS) remains an unelucidated area that needs further attention. In this study, we aimed to analyze the patterns of neurologic involvement and evaluate their association with other clinical features. Methods 800 patients with BS diagnosis according to The International Criteria for Behcet’s Disease (ICBD) followed in our center were retrospectively screened and 55 patients with central nervous system involvement and 20 patients with non-vascular BS were enrolled in this study. These patients were classified as sinus vein thrombosis (SVT), parenchymal involvement, and mixed (SVT and parenchymal). These groups were compared in terms of demographic and clinical features. Results Thirty-two (58,2%) of the patients were male. Distribution of neurologic involvement were as follows; 22 (40%) with isolated sinus vein thrombosis (SVT), 25 (%45) with isolated parenchymal, and 8 (%15) with mixed pattern. Central nervous involvement was the diagnostic feature of BS in 25 patients. Headache was the most common symptom followed by neurologic deficits and dizziness (44%, 25%, 16%). The most common sites of involvement were subcortical and periventricular areas followed by brainstem (63% vs 37%). In SVT group, positive family history of BS (p < 0.001) and previous deep vein thrombosis (DVT) (p:0.004) were found higher. Smoking history, erythema nodosum, cardiac and pulmonary involvement were significantly higher in parenchymal group (p < 0.001) (Table 1). Conclusion Although brainstem involvement is considered as more characteristic in BS, white matter and periventricular involvement were also found as common in our study. Notably, these subgroup analyses may suggest the speculation that BS shows clusters even within the neuro-vascular involvement. Disclosure R. Yıldırım: Other; Reşit Yıldırım is a member of Rheumatology journal as an associate editor. M. Dinler: None. A.E. Arslan: None. N.S.Y. Bilge: None. T. Kaşifoğlu: None.

Clinical value of soluble urokinase-type plasminogen activator receptor (SuPAR) as a biomarker of disease activity in Behcet’s disease

Background: Behçet's disease (BD) is a chronic multisystemic vasculitic disease of unknown pathogenesis. Diagnosis and disease activity measurement has always been a challenge. Soluble urokinase plasminogen activator receptor (suPAR) is showing an increased utility as a promising diagnostic and prognostic inflammatory biomarker in different systemic inflammatory disorders. Objectives: To study the clinical value of plasma Soluble urokinase plasminogen activator receptor (suPAR) in patients with Behçet's disease, and if it can be used as a measure to disease activity. Methods: According to the International Criteria for Behcet's Disease (ICBD), fifty adult Behçet's disease patients were enrolled in the study, and forty healthy adult subjects were included in a case-control study. An enzyme-linked immunosorbent assay was used to obtain quantitative data. Results: The suPAR level was not statistically different between the patients with BD patients and the controls. No correlation was found between suPAR levels and disease activity (BDFAC). Conclusion: Very few studies analyzed the clinical value of suPAR level in patients with BD and its correlation with disease activity. Our results show that suPAR does not seem to play a role in the disease mechanism of BD.

Evaluation of different classification criteria in children with Behcet disease: results from a single referral center

ABSTRACT Objectives Behcet Disease (BD) is a systemic vasculitis, first described with a triad of oral aphthous ulcers, genital ulcers, and uveitis. The authors aimed to share the clinical properties and utilities of three distinct classification criteria for BD in this study. Methods This case–control study was conducted in pediatric BD patients, diagnosed between January 2012 and July 2019. The control group included 53 children with other rheumatic disorders. Sensitivity and specificity for International Study Group (ISG), International Criteria for BD (ICBD), and pediatric criteria for BD (PEDBD) criteria were tested. Results The mean age at symptom onset and diagnosis of the 16 BD patients (6 females, 10 males) were 11.2 ± 3.6 and 13 ± 3.1 years, respectively. The sensitivity and specificity of ISG criteria were 87.5% and 100%. Furthermore, ICBD criteria had a sensitivity and specificity of 93.7% and 98.1%, whereas the authors found sensitivity and specificity as 93.7% and 96.2% for PEDBD. Conclusion ICBD and PEDBD reached higher sensitivity for pediatric BD diagnosis and ICBD had the highest specificity. The authors speculate that the utilization of ICBD may provide early diagnosis of BD in childhood, prevent related morbidities and misdiagnosis.

PERIPHERAL NEUROPATHY IN BEHÇET'S DISEASE: CLINICAL AND NEUROPHYSIOLOGICAL STUDY

Background: Behcet’s disease (BD) is a chronic idiopathic relapsing condition, whereas multiple organs are affected as a sequential effect of the vascular and inflammatory process. Based on the neurophysiological studies, 20-40% of BD patients may have silent neurological involvement (SNI) with no or very mild clinical manifestations. Objectives: Assessing clinically and electro physiologically the prevalence of symptomatic and asymptomatic peripheral neuropathy (PN) among BD patients along with the potential predictors of PN among BD patients. Patients and Methods: All patients with clinically established BD according to the International Criteria for Behcet's Disease (ICBD) were involved in the current study. All patients were submitted to clinical evaluation, neuropathy symptom score (NSS) along with electrophysiological nerve conduction studies including sensory and motor nerve conduction assessment. Results: An overall 30 participants who fulfilled the inclusion criteria were enrolled in the study. There were 22 (73.3%) males and 8 (26.7%) females with a mean age of 37.9±8.31 years. Based on the electrophysiological studies, 16 (53.3%) patients had the evidence of peripheral neuropathy in the terms of mixed (23.3%), motor (6.6%), and sensory (23.3%) nerve affection. The most affected peripheral nerve was sural nerve (46.6%) succeeded by peroneal (43.3%) and ulnar (20%) nerves. The univariate regression model showed that the age of the patients and the duration of the disease were independent predictors of peripheral neuropathy. Conclusion: PN is a considerable complication of BD, whereby health care providers should employ a routine follow up protocol for early detection of even the subclinical PN.

PERIPHERAL NEUROPATHY IN BEHÇET'S DISEASE: CLINICAL AND NEUROPHYSIOLOGICAL STUDY

Background: Behcet’s disease (BD) is a chronic idiopathic relapsing condition, whereas multiple organs are affected as a sequential effect of the vascular and inflammatory process. Based on the neurophysiological studies, 20-40% of BD patients may have silent neurological involvement (SNI) with no or very mild clinical manifestations. Objectives: Assessing clinically and electro physiologically the prevalence of symptomatic and asymptomatic peripheral neuropathy (PN) among BD patients along with the potential predictors of PN among BD patients. Patients and Methods: All patients with clinically established BD according to the International Criteria for Behcet's Disease (ICBD) were involved in the current study. All patients were submitted to clinical evaluation, neuropathy symptom score (NSS) along with electrophysiological nerve conduction studies including sensory and motor nerve conduction assessment. Results: An overall 30 participants who fulfilled the inclusion criteria were enrolled in the study. There were 22 (73.3%) males and 8 (26.7%) females with a mean age of 37.9±8.31 years. Based on the electrophysiological studies, 16 (53.3%) patients had the evidence of peripheral neuropathy in the terms of mixed (23.3%), motor (6.6%), and sensory (23.3%) nerve affection. The most affected peripheral nerve was sural nerve (46.6%) succeeded by peroneal (43.3%) and ulnar (20%) nerves. The univariate regression model showed that the age of the patients and the duration of the disease were independent predictors of peripheral neuropathy. Conclusion: PN is a considerable complication of BD, whereby health care providers should employ a routine follow up protocol for early detection of even the subclinical PN.

Characteristics of Behcet's Disease in the American Southwest

ObjectiveThe goal of this study was to determine the characteristics of Behçet's disease (BD) in the American Southwest. Material and methodsThis was a cross-sectional study of BD patients clinically encountered during a 2-year period. All subjects fulfilled the International Study Group criteria (ISG) or International Criteria for Behcet's Disease (ICBD). Age, gender, clinical characteristics, substance use, and HLA-B51 status were determined. Results63 patients (female: male ratio: 4.7:1) fulfilled ISG criteria and 76 the ICBD criteria (estimated prevalence of 8.9–10.6 per 100,000). 84.1% (53/63) were initially diagnosed with non-BD primary diagnoses including inflammatory arthritis (15.9%), fibromyalgia (7.9%), vasculitis (7.9%), or systemic lupus erythematosus (7.9%). Common BD manifestations were oral aphthous ulcers (100%), acneiform lesions (69.8%), genital aphthous ulcers (61.9%), papulopustular lesions (52.4%), pseudofolliculitis (42.9%), inflammatory arthritis (41.3%), anterior uveitis (23.8%), posterior uveitis (15.9%), pathergy (15.9%), deep vein thrombosis (14.3%), non-ocular vasculitis (11.1%), erythema nodosum (7.9%), arterial thrombosis (6.3%), and retinal vasculitis (1.6%). BD ethnic proportions were 49.2% Hispanic American (HA), 31.7% European-American (EA), 14.3% Native American (NA), and 1.7% Silk Road. HLA-B51 was present more in NA (89.0%, p = 0.02) and HA (74.2%, p = 0.02) compared to EA (42.1%). Therapy of BD was conventional, except for the frequent use of hydroxychloroquine. ConclusionsBD is common in the American Southwest with a prevalence of 8.9–10.6 cases per 100,000. BD patients are commonly initially diagnosed with alternative primary conditions. Hydroxychloroquine may be an effective alternative therapy for BD. This is one of the first reports of BD in HA and NA populations.

Comparison of the performance of three classification criteria for Behçet's Disease in a single centre cohort from South India

Background: A new International Paediatric criteria for Behcet's disease (PEDBD) 2015 has been introduced in the presence of existing revised International Criteria for Behcet's Disease (ICBD) and the International Study Group (ISG) criteria. In this study, we compared the performance of the new PEDBD criteria with the existing criteria in the pediatric group of Behcet's disease. Methods: In this retrospective study eligible patients were identified from a database and their already recorded characteristics were analyzed. A total of 25 patients who visited the pediatric rheumatology clinic were included in the cohort. Performance of the ISG, revised ICBD, and PEDBD 2015 was evaluated in terms of sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), negative likelihood ratio (NLR), positive likelihood ratio (PLR), diagnostic odds ratio (DOR), accuracy, and Youden's index (YI). Results: The revised ICBD has the maximum sensitivity (88.24%) when compared to PEDBD (64.71%) and ISG (47.05%). ISG had 100% specificity, with a comparable value in revised ICBD (87.5%) and PEDBD (87.5%). PPV was similar in all the three criteria with ISG 100%, revised ICBD 93.75%, and PEDBD 91.67%. NPV in revised ICBD was 77.78%, ISG was 47.06%, and PEDBD 53.85%. PLR of revised ICBD was 7.06 and PEDBD was 5.18. NLR was detected to be 0.53 in ISG as it was 0.40 in PEDBD and 0.13 in revised ICBD. The DOR in revised ICBD was 52 and PEDBD was 12.83. YI was 0.47 in ISG, 0.76 in revised ICBD, and 0.52 in PEDBD. The accuracy was best for revised ICBD 88% and PEDBD 72%. Conclusion: Revised ICBD criteria hold a higher statistical significance in the diagnosis of the Behcet's disease even in the pediatric age group. PEDBD has a comparable sensitivity and PPV although the NPV and specificity are very low in comparison to the other criteria. It was observed that oral aphthosis, despite being the most common manifestation in the Behcet's disease pediatric group, other features, mainly vascular involvement was the most common presenting complaint.

Comparison of International Study Group Criteria and International Criteria for Behcet's Disease in the Azeri population of Iran

Introduction: Behcet's disease (BD) is a chronic systemic inflammatory disease affecting multiple organs and systems such as mucosa, skin, eye, joints and cardiovascular, nervous and gastrointestinal systems. Various criteria are proposed for the assessment of BD. This study was designed to compare performance of two internationally collaborated criteria for diagnosis of BD, namely International Study Group (ISG) criteria and International Criteria for Behcet's Disease (ICBD) in the Azeri population of Iran. Methods: In a descriptive analytical study, 859 consecutive patients with one of the major clinical manifestations of BD were included. All patients were examined and evaluated by an expert rheumatologist and diagnosis was confirmed clinically. All patients were assessed by ISG and ICBD criteria. Finally, sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of both criteria were calculated. Results: We included 859 patients in this study. BD was diagnosed in 211 patients. Sensitivity, specificity and accuracy of ISG criteria were 64.9%, 100%, and 91.4%, respectively. Sensitivity, specificity and accuracy of ICBD criteria were 94.7%, 99.6% and 98.5%, respectively. Conclusion: Based on the finding of the present study, sensitivity and accuracy of ICBD criteria for diagnosis of BD in Azeri population are higher than ISG criteria. So we propose to use ICBD criteria for the evaluation of patients with suspected BD to decrease the rate of missed diagnosis.

Performance of classification criteria for Behcet's disease in an Egyptian cohort

Background: The revised Japanese criteria, the International study group (ISG), the International criteria for Behcet's disease (ICBD) 2006, and the revised ICBD 2010 are frequently used for the classification of Behcet's Disease (BD). In this study we evaluated the performance of these criteria sets in Egyptians. Methods: A total of of 461 Egyptian patients over 5 years were studied. It included 256 patients classified as BD based on expert opinion and 205 patients with other autoimmune and/or autoinflammatory diseases with symptoms similar to BD. Performance of the revised Japanese criteria, ISG, ICBD 2006, and the revised ICBD 2010 was evaluated evaluated in terms of sensitivity, specificity, negative predictive value (NPV), negative likelihood ratio (NLR), positive predictive value (PPV), positive likelihood ratio (PLR), diagnostic odd ratio (DOR), and Youden's index (YI). Results: ICBD 2010 carried the highest sensitivity (98.83%), NPV (98.48%), DOR (1645), and YI (0.94) with lowest NLR (0.01). On the other hand, ICBD 2006 and ISG were very specific (99.51%, 99.41%, respectively) with PPV (99.49%, 99.40%) and PLR (155.35, 126.33), respectively. Conclusions: ICBD 2010 is a very good criteria set to be used in Egyptian BD patients based on its very high sensitivity, accepted specificity, and power of discrimination that enables early patients classification, management, and prognosis.

Behcet’s Disease Criteria

Objective: From 1946 to 2014, 16 different sets of Classification/Diagnosis criteria, from different countries, have been made for Behcet’s Disease. Among them, there are two International criteria sets, the International Study Group (ISG) criteria in 1990 by the collaboration of 7 countries, and the International Criteria for Behcet’s Disease (ICBD) in 2010 by the collaboration of 27 countries. The aim of this study is to compare the performance of them, in new patients and controls, from the Iran registry of Behcet’s Disease.Methods: Patients (1323) and controls (2438) are consecutive patients, seen from 2010 to 2016. The diagnosis was clinical and by expert opinion. Sensitivity, specificity, accuracy, optimization, efficiency, positive predictive value, negative predictive value, positive likelihood ratio, negative likelihood ratio, diagnostic odds ratio, and Youden’s index were calculated.Results: The sensitivity of ISG was 64% versus 96.9% ICBD (a difference of 32.9%), while specificity was 99.9% versus 97.4% (a difference of 2.5%), and accuracy 87.3% vs. 97.2% (a difference of 9.9%). The optimization (100- difference between sensitivity and specificity. The best score is 100) was 64.1 versus 99.7 (a difference of 35.6). The efficiency ((optimization/100) × accuracy. The best score is 100) was 56 versus 96.9 (a difference of 40.9).Conclusion: The differences for all the items was statistically highly significant, even for the specificity, although numerically the difference was small, just 2.5%. However, clinically, the difference was not relevant. The performance of ICBD is much higher than that of ISG. The ICBD criteria have a much higher sensitivity, accuracy, optimization, and efficiency, with clinically, approximately the same specificity.

HLA-B5/51 GENOTYPE: AN ASSOCIATION WITH THE CLINICAL MANIFESTATIONS OF BEHCET’S DISEASE

Objective: to estimate the contribution of HLA-B5/51 genotype to the clinical manifestations and risk of Behcet’s disease (BD) in two ethnic groups. Subjects and methods. 146 BD patients fulfilling the International Criteria for BD (ICBD) were divided into two ethnic groups: 1) 86 patients from Dagestan (representatives of 8 ethnic nationalities in this region) with mean age 30.7±9.6 years; disease duration – 8.8±10.1 years; 2) 60 ethnic Russian patients, nonresidents of Dagestan with mean age 32.9±11.1 years; disease duration – 11.2±10.1 years. All patients were examined at the V.A. Nasonova Research Institute of Rheumatology in 1990 to 2014. HLA class I antigens were typed by a microlymphocytotoxic technique using a Gisans anti-leukocyte sera kit (Saint Petersburg). Results. HLA-B5/51 was detected in 87 (59.6%) patients, much more often in men than in women (70 and 38%, respectively; p<0.01). Genital ulcers and erythema nodosum were significantly more common in HLA-B5/51-positive Dagestani (87.3 and 57%) than in HLA-B5/51-negative ones (56.5 and 26%; p=0.0019 and р=0.01; respectively). There were no significant differences in these signs in the Russian group of patients with BD depending on the presence of this allele. In HLA-B5/51-positive male Dagestani patients with BD, the risk of erythema nodosum was twice as high as that in HLA-B5/51-negative patients (p=0.054). In HLA-B5/51 female Dagestani carriers, the risk of genital ulcers and generalized uveitis proved to be 3.5 (p=0.057) and 2.7 times higher than that in HLA-B5/51 noncarriers. Frequency of HLA-B5/51 was 73.2% among the Dagestanis and 40% among the Russians. Furthermore, this investigation revealed HLA-B5/51 carriage mainly in the male BD patients. Therefore, in addition to ethnicity, gender should be borne in mind when analyzing the clinical associations with HLA-B5/51.

BEHCET'S DISEASE IN CHILDREN: RUSSIAN EXPERIENCE

The paper presents the clinical characteristics of Behcet's disease (BD) in Russian children, generally accepted criteria for BD, and diagnosis statement. The investigation is based on an observation of 17 children who met the international criteria for BD and were treated at the V.A. Nasonova Research Institute of Rheumatology in 2004 to 2013. One-third of children had a compromised family history; about 30% of the patients were ethnic Russian. The age at BD onset ranged from infancy to 16 years. The interval between disease onset and diagnosis was 1 to 10 years. The clinical symptoms at onset represented mostly frequently aphthous stomatitis and its accompanying fever and much less frequently aphthae of the genitalia. Furtherthe pathological process involved other organs and systems. In advanced stage of disease, there was already a clinical syndrome encompassing aphthous stomatitis, a genital ulcerative process, and lesions of the eye, skin, gastrointestinal tract, and less often central nervous system. The manifestations of the disease in different combinations can be concurrent with each other, showing a mosaic pattern. Treatment of children with BD included glucocorticoids, colchicine, azathioprine, and, if clinically indicated, tumor necrosis factor-α inhibitors. Their efficacy is discussed. The pediatric aspects of BD, which are presented in this paper, are first published in Russian medical press

Adult Behcet's disease in Iran: analysis of 6075 patients.

This 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%). Patients 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. Males 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%. Results are near the nationwide surveys from Japan, China, Korea and Germany.

The relationship between plasma homocysteine level and HLA‐B51 in patients with Behcet's disease: a case‐control study

Various coagulation disorders have been reported to explain hypercoagulability state in Behcet's disease (BD). A possible negative association between human leukocyte antigen (HLA)-B51 and increased homocysteine level has been suggested in a previous report from Iranian patients with BD. The aim of this study was to find any possible relationship between plasma homocysteine levels and HLA-B51. In a case-control study, BD patients (fulfilling the new International Criteria for BD) and controls (who had similar clinical symptoms but BD was clinically excluded in them) were included. Mean plasma homocysteine levels measured by enzyme-linked immunosorbent assay in HLA-B51 positive and negative individuals both in patients and controls were compared by t-test, Mann-Whitney test and analysis of variance (F-test). Ninety-six BD patients and 152 controls were recruited. There was no significant difference between HLA-B51 positive and negative individuals either in the mean plasma homocysteine levels (13.59 ± 9.03 vs. 12.95 ± 4.98 μmol/L, P = 0.514), or in the prevalence of hyperhomocysteinemia (17% vs. 21.4%, P = 0.504). This was true both for BD and control groups. In HLA-B51 positive and negative BD patients, mean plasma homocysteine levels were 14.29 ± 12.02 and 12.62 ± 4.79 μmol/L, respectively (P = 0.33), and the prevalence of hyperhomocysteinemia was 20.8% versus 19.5% (P = 0.55). In the control group, the mean plasma homocysteine levels in HLA-B51 positive and negative individuals were 12.85 ± 4.28 and 13.14 ± 5.10 μmol/L, respectively (P = 0.794), and the prevalence of hyperhomocysteinemia was 13% versus 22.1% (P = 0.23). The difference was non-significant regarding sex (P > 0.71) and disease activity (P > 0.31). In contrast to our previous report, we found no relationship between plasma homocysteine levels and HLA-B51 in this study, either in BD or in the control group.

Validation of the revised International Criteria for Behcet’s Disease (ICBD) in Iran

The first internationally agreed criteria for Behcet's disease were the International Study Group (ISG) criteria. It had very high specificity, but lacked good sensitivity, missing an important subset of patients. The International Criteria for Behcet's Disease (ICBD) were created in 2006 to overcome this lack of sensitivity. It was revised in 2010. The objective of this study was to evaluate the performance of the revised International Criteria for Behcet's Disease (rICBD) in Iran. In this study, the ISG and ICBD were evaluated and compared to the rICBD. All patients from the Behcet's Disease Registry (7,011) and controls (5,226), up to March 2013, entered the study. The diagnosis was clinical, by expert opinion. Sensitivity, specificity, and accuracy were calculated for ISG, ICBD, and rICBD. A 95% confidence interval (95%CI) was calculated for percentages. For ISG, the sensitivity was 77.5% (95%CI = 76.5-78.5). It was 98.3% for ICBD (95%CI = 98.0-98.6) and 96.8% for rICBD (95%CI = 96.4-97.2). Specificity was 99.2% (95%CI = 99.0-99.4) for ISG, 96.2% for ICBD (95%CI = 95.7-96.7), and 97.2% for rICBD (95%CI = 96.8-97.6). Accuracy was 86.7% (95%CI = 86.1-87.3) for ISG, 97.4% for ICBD (95%CI = 97.1-97.7), and 97.0% for rICBD (95%CI = 96.7-97.3). In Iranian patients, ICBD has 20.8% and rICBD 19.3% higher sensitivity than ISG. Although the specificity was lower than ISG by 3% for ICBD and 2% for rICBD, the accuracy was higher respectively by 10.7 and 10.3%. ICBD has by far better performance than ISG. The difference was even more prominent in Iranian patients than for the ICBD cohort of patients and controls.

Clinical features of Behcet's disease in patients without oral aphthosis

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 6821 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.