International Criteria For BD Criteria Research Articles

Performance of diagnostic criteria in pediatric Behçet's disease.

Behçet's disease is an inflammatory vasculitic disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular findings, and multiple organ involvement. Mucocutaneous findings are the most common symptoms. The most used diagnostic criteria are International Criteria for BD (ICBD), International Study Group (ISG) criteria and pediatric Behçet's disease criteria (PEDBD). Although diagnostic criteria have been defined, the diagnosis is still difficult due to clinical findings developed in pediatric patients. The aim of this study was to evaluate the clinical findings, phenotype characteristics, sensitivity and specificity of diagnostic criteria, and the course of pediatric Behçet's disease (BD). We evaluated retrospectively the files of 67 (29M/38 F) patients diagnosed with BD according to expert opinion. The patients were reclassified according to ISG, ICBD, and PEDBD criteria. The control group consisted of a total of 69 patients with BD-mimicking diseases or presenting at least one major BD sign followed at the same center. Sensitivity and specificity were evaluated for the criteria. The mean age of diagnosis was 13.38 ± 3.2years. There were oral aphthous ulcers in 98.5%, genital ulcers in 68.7%, skin lesions in 31.3%, vascular involvement in 17.9%, neurological involvement in 11.9%, positive pathergy test in 33.8%, and positive HLA-B51 in 57.1% of patients. The sensitivity of ICBD, ISG and PEDBD criteria was 88.1, 43.3, and 37.3%, respectively. The specificity of ICBD, ISG, and PEDBD criteria was 100%. Diagnosis of BD in childhood is still difficult. ICBD criteria have the highest sensitivity among the diagnostic criteria. These criteria can also be used in childhood.

Antineutrophil Cytoplasmic Antibody Positivity Is Associated with Vascular Involvement in Behçet's Disease.

PurposeWe investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up.Materials and MethodsWe retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis.ResultsAmong the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up.ConclusionANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.

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.

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.

Behçet's disease in the United States: A single center descriptive and comparative study.

Behçet's disease is heterogeneous with clinical variability across ethnicities and geographic locations. The goal of this study was to analyze the clinical characteristics of our multi-ethnic Behçet's disease cohort at the University of Michigan. A detailed patient characterization was performed. Differences in disease characteristics between men and women, and between patients fulfilling the International Criteria for Behçet's Disease (ICBD) and the International Study Group criteria (ISG) were determined in our cohort. A total of 114 patients with a male to female ratio of ~ 1:4 were included. All patients met the ICBD criteria, including 76 who also met the ISG criteria. Over 95% of patients had recurrent genital ulcers, which is higher than generally reported. Retinitis was 5.3 times more likely in men than in women (p=0.009), and arthralgia was 3.3 times more likely in women than men (p=0.048). When comparing cohorts derived from the two different criteria, the ISG cohort had more skin manifestations (OR=3.3, p=0.0006). Acneiform lesions were associated with ~8 times higher odds of developing retinitis in our patients (p=0.0008), and superficial thrombophlebitis was associated with a trend for higher odds of developing uveitis (OR=4.1, p=0.057). Using the ICBD criteria, 38 additional patients were identified compared to only using the ISG criteria. Of these patients, 28 presented with only mucosal ulceration with or without joint involvement. We characterize Behçet's disease in a multi-ethnic cohort from North America. Using ICBD criteria in the United States significantly increases the likelihood of identifying Behçet's disease, particularly in patients with isolated mucosal involvement who constitute a substantial subset of patients in this region.

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.

Auditory involvement in Behcet's disease: relationship with demographic, clinical, and therapeutic characteristics.

The study objective was to evaluate the occurrence of sensorineural hearing loss (SNHL) in patients with Behcet's disease (BD), looking at potential correlations with specific demographic, clinical, and therapeutic features. Forty-four consecutive patients (15 males, 29 females) fulfilling the International Study Group (ISG) and/or the International Criteria for Behçet's Disease (ICBD) were enrolled. The endpoints of the study consisted in identifying a deflection of at least 25dB on pure-tone audiometry and performing statistical analysis to evaluate demographic, clinical, or therapeutic differences between patients with and without SNHL. Our patients showed a mean age ± SD of 45.43±14.05years; a mean age at disease onset ± SD of 31.54±15.53years; a disease duration ± SD of 13.89±9.15years. SNHL was highlighted in 28 (63%) patients representing the fourth most frequent clinical manifestation in our group of patients. Otologic involvement was significantly more frequent among subjects fulfilling ISG criteria than in patients fulfilling ICBD criteria (p=0.04). Regarding correlations with BD manifestations, SNHL was significantly associated with cutaneous plus articular involvement (p=0.013). Conversely, detached analysis of articular and skin manifestations led to no significant differences (p=0.085 and p=0.067). No further significant correlations were found between SNHL and BD clinical features or previous or concomitant treatments. Hearing loss was the fourth most common clinical feature in our patients and probably represents an underrated aspect of BD. Hearing impairment was significantly associated with cutaneous plus articular involvement, suggesting the importance of an otologic evaluation in such patients.

Paediatric Behçet's disease: a UK tertiary centre experience.

There are currently limited data regarding paediatric Behçet’s disease (BD), particularly in the UK. We describe the clinical spectrum, treatment and outcome of BD, and explore the relative sensitivities of the criteria for the diagnosis of BD in a UK paediatric cohort. Single retrospective case note review of children with a clinical diagnosis of BD presenting between 1987 and 2012. Demographics, clinical features, treatment and outcomes were recorded. The sensitivities of the International Study Group (ISG) and International Criteria for BD (ICBD) criteria were explored. BD disease activity was calculated using the Behçet’s Disease Activity Index (BDAI). Forty-six patients (22 male) were identified. Median age of onset was 4.87 (0.04–15.71) years; median time to diagnosis was 3.74 (0.25–13.48) years. Clinical features were recurrent oral ulceration (97.8 %), recurrent genital ulceration (73.9 %), gastrointestinal (58.7 %), musculoskeletal (47.83 %), cutaneous (23.9 %) involvement and uveitis (2 %). Recurrent genital ulceration was more common in female patients (P = 0.044). Thirty-seven patients (80.4 %) fulfilled the ICBD criteria; only 12 patients (26.1 %) fulfilled the ISG criteria. BDAI score at diagnosis was 7/20 (0–10/20) and significantly decreased to 5/20 (0–9/20) (P < 0.0001) at latest follow-up. The commonest systemic treatment was colchicine (76.1 %); anti-TNFα treatment was reserved for severe cases (15.5 %). Paediatric BD in the UK may present very early in life, sometimes with a family history, and with a low incidence of ocular involvement. Diagnostic delay is common. The majority of our patients required systemic therapy; anti-TNFα was reserved for severe cases and has largely superseded the use of thalidomide.Electronic supplementary materialThe online version of this article (doi:10.1007/s10067-016-3187-z) contains supplementary material, which is available to authorized users.

The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria

Behçet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment. An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, 'leave-one-country-out' cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4-95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9-92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.

Diagnosis/Classification Criteria for Behcet's Disease.

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG.