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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.

Daniel Kastner, Elaine Remmers and colleagues perform an association study of Behçet's disease based on dense genotyping of immune-related loci. They identify new association signals near genes involved in host response to microbial exposure and extend evidence for shared susceptibility loci with Crohn's disease and leprosy. We analyzed 1,900 Turkish Behçet's disease cases and 1,779 controls genotyped with the Immunochip. The most significantly associated SNP was rs1050502, a tag SNP for HLA-B*51 . In the Turkish discovery set, we identified three new risk loci, IL1A – IL1B , IRF8 , and CEBPB – PTPN1 , with genome-wide significance ( P < 5 × 10 −8 ) by direct genotyping and ADO – EGR2 by imputation. We replicated the ADO – EGR2 , IRF8 , and CEBPB – PTPN1 loci by genotyping 969 Iranian cases and 826 controls. Imputed data in 608 Japanese cases and 737 controls further replicated ADO – EGR2 and IRF8 , and meta-analysis additionally identified RIPK2 and LACC1 . The disease-associated allele of rs4402765, the lead marker at IL1A – IL1B , was associated with both decreased IL-1α and increased IL-1β production. ABO non-secretor genotypes for two ancestry-specific FUT2 SNPs showed strong disease association ( P = 5.89 × 10 −15 ). Our findings extend the list of susceptibility genes shared with Crohn's disease and leprosy and implicate mucosal factors and the innate immune response to microbial exposure in Behçet's disease susceptibility.

Background A strong correlation was previously found between mean platelet volume (MPV), red blood cell distribution width (RDW), and the severity of signs and symptoms in patients suffering from inflammatory and autoimmune diseases. The current study evaluated these correlations in patients with Behçet’s disease (BD) as well the relationship between MPV and RDW and disease activity score on the Iranian Behçet’s Disease Dynamic Activity Measurement (IBDDAM). Methods This cross-sectional study included 319 patients with BD for whom demographic and epidemiological data, IBDDAM scores, and duration of illness was recorded. Blood samples were then obtained and the relationships between their disease status and manifestations and their laboratory parameters were evaluated with statistical models to find possible correlations. Results Our analysis showed a significantly higher RDW in patients with BD who had ocular manifestations ( p  < 0.001) and oral aphthae ( p  = 0.004). Patients with active BD had higher RDW ( p  < 0.001) and MPV ( p  < 0.001) in comparison to those with currently inactive BD. Similarly, patients who had any type of ocular manifestation had higher RDW ( p  < 0.001) and MPV ( p  < 0.001). Regression analyses identified a statistically significant model for the effect of RDW and MPV in predicting active BD status ( p  < 0.001), as well as its significant relationship with active ocular manifestations ( p  < 0.001). Conclusion BD was found to be associated with an increase in MPV and RDW, particularly during active phases. RDW and MPV were also found to have predictive value for screening to detect BD activity and its ocular complications.

Behçet’s disease (BD) is a chronic disorder that involves multiple organs and is pathologically considered as a form of vasculitis. The current study aims to assess the metric properties of platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) in assessing BD disease activity. Three-hundred-nineteen patients with BD were enrolled in this cross-sectional study. Demographic and epidemiological data, including IBDDAM, time since the onset, and medication and manifestation history were recorded. Complete blood counts (CBC), NLR, and PLR were assessed by analyzing blood samples. On the last visit, patients were assessed for active manifestations of disease. IBDDAM and ocular IBDAAM scores were calculated for activity of disease in each patient. Both PLR and NLR were higher in patients with active BD (Mann–Whitney U test, p-value < 0.05). Patients with active ocular manifestation had significantly higher NLR and PLR (Mann–Whitney U test, p-value < 0.05). These ratios, however, were not associated with other active BD manifestations. A value of NLR > 2.58 had 46% sensitivity and 85% specificity for the diagnosis of active ocular manifestations (AUC: 0.690). NLR had a significant, though, weak positive correlation with IBDDAM (Spearman’s rho = 0.162; p-value < 0.05) and ocular IBDDAM (Spearman’s rho = 0.159; p-value < 0.05). Active Behçet’s presented with higher NLR and PLR ratios; however, there was only a modest correlation between NLR and BD activity (IBDDAM score). Also, NLR and PLR have significant relationship with ocular features of BD patients.

Background The diagnosis of Behçet disease (BD) is challenging in many cases. The purpose of this study was to describe the clinical characteristics of patients at a referral BD clinic. Methods In a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018–August 2019. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences. Results A total of 238 patients satisfied the inclusion criteria. Forty patients (16.8%) were finally diagnosed with BD. Ocular and genital lesions were significantly more prevalent in the BD group. A positive pathergy test and HLA-B51 were also significantly more common in BD. However, oral lesions, articular involvement, and gastrointestinal manifestations were similar between groups. Also, patients with BD were significantly more likely to have multi-organ (≥2 organ systems) involvement. Conclusions Being the first study to evaluate the clinical characteristics of patients who are visited at a referral BD clinic and are believed to have a high probability of Behçet, the results of this study are important from an epidemiological standpoint. Also, the findings of this study could be used by referral Behçet clinics, which evaluate and diagnose patients with a high pretest probability and atypical presentations of BD on a daily basis. The alternative diagnoses established in this study could be used as the list of the most common differential diagnoses for Behçet’s disease.

Pathergy test (PT) is used for the diagnosis of Behcet’s disease (BD). It is a criterion in many classification/diagnosis criteria. PT is mainly seen in BD but can be seen in other conditions too. PT has been reported with high frequency from most countries along the Silk Road. The sensitivity of pathergy phenomenon (PP) is declining over the time. The aim of this study was to look for the diagnostic value of PT in the past and at the present time. The BD registry (Rheumatology Research Center, Tehran University of Medical Sciences) has the data of 6,607 BD and 4,292 control patients. Patients and controls were divided in four groups of 1,650 BD and 1,073 controls. Sensitivity, specificity, positive and negative predictive value (PPV-NPV), positive and negative likelihood ratio (PLR-NLR), diagnostic odds ratio (DOR), and Youden’s index (YI) were calculated for each group. The first and the fourth quartiles were compared. Sensitivity of PT decreased from 64.2% (first quartile) to 35.8% (fourth quartile). Specificity improved from 86.6% to 98.4%. PPV improved from 82.7% to 95.7%. NPV decreased from 82.7% to 60.5%. PLR improved from 4.8 to 22.4, while NLR deteriorated from 0.41 to 0.65. DOR improved from 11.6 to 34.3. Yuden’s index worsened from 0.5 to 0.34. Although sensitivity of PP decreased, the increase of specificity is a plus value for diagnosis. As a result, PPV, PLR, and DOR improved, while NPR, NLR, and YI deteriorated. Although the pathergy test lost its sensitivity during the past 35 years, it has not lost its value as a diagnostic test, improving many of its characteristics. In a practical view, the chances of getting a positive test have decreased over the time. However, a positive test is rather the synonym of Behcet’s disease, with a probability of 98.4%.

Behçet’s Disease (BD) pathogenesis remains unclear, but some genetic loci and environmental factors are proposed to play a role. Here, we investigate the association of the endoplasmic reticulum aminopeptidase-1 ( ERAP1 ) gene variants and HLA-B*51 with BD susceptibility and clinical manifestations in Iranian patients. In the study, 748 BD patients and 776 healthy individuals were included. The MGB-TaqMan Allelic Discrimination method was used to genotype 10 common missense single nucleotide polymorphisms (SNPs) and one intronic SNP in the ERAP1 gene region. We found no significant association between the 11 SNPs and BD in allelic and genotypic association tests. However, rs30187 showed the strongest association with BD in the recessive genotype model of the risk T allele in HLA-B*51 carriers. Although this became insignificant after correcting for multiple comparisons, the homozygous rs30187 risk allele genotype (TT) increased disease susceptibility in HLA-B*51 carriers in epistasis analysis, and the rs30187 TT recessive genotype showed a significant association with risk of cardiac involvement in the all patients and articular involvements in HLA-B*51 positive patients. Our findings suggest that gene-gene interactions between HLA-B*51 and ERAP1 variants is important for BD development, however, ERAP1 variants which interact with HLA-B*51 may differ among disease phenotypes or populations.

Background Low back pain (LBP) is a major contributor to chronic pain and disability. The purpose of this study was to evaluate health-care access and utilization among patients with LBP in Iran. We also sought to study the pattern and characteristics of care-utilization behavior in these patients. Methods Data from the Community Oriented Program for Control of Rheumatic Diseases (COPCORD) were used for this study. Three cities (Zahedan, Sanandaj, Yazd) were selected to represent the Iranian population, with different socioeconomic status and ethnic, cultural, and religious background. Demographic data, acute or chronic LBP, disability index, and utilizing care from conventional medicine (CM), allied health providers (AHP), and complementary and alternative medicine (CAM) providers were recorded. Results Of 9101 patients, 38.6% reported LBP. Only 3.3% did not utilize care of any kind, 66.7% referred to CM providers, 20.8% to AHP, and 9.2% to CAM care. Health-care utilization was higher in female patients, older age, higher education, and higher disability index. Conclusions The findings of this study indicate a high rate of health-care utilization among patients with LBP in Iran. CM is the most prevalent health-care resource sought by patients. These findings could be used as a framework in developing more efficient health-care programs according to the needs of specific populations.

Background Behcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis. Methods We have retrospectively reviewed all patients in Iran Behcet’s Disease Registry and reported those with osteonecrosis. Patients’ medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed. Results Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated. Conclusions ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.

Background Behcet’s disease (BD) as a chronic inflammatory condition that affects the eyes, skin, central nervous system, gastrointestinal tract and vessels. According to the literature, the exact value of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) in predicting active manifestations of BD remains controversial. In this study, we aim to assess and compare values of ESR and CRP between BD patients with active/inactive BD and active/inactive manifestations of the disease. Moreover, we try to determine the predictive value of ESR and CRP for disease activity. Methods Participants (n = 514) were drug-naïve BD patients; Based on last two visits, ESR and CRP values, disease activity, and active manifestations were recorded. The Man-Whitney U test measured the associations, and the binomial logistic regression evaluated the predictive value of ESR and CRP for active disease and each active manifestation. The sensitivity and specificity and the area under the curve (AUC) for each model were determined using receiver operating characteristic curves (ROC). Multiple regressions were run to predict BD activity score from ESR and CRP. Result Patients with active oral, genital, joint and dermal manifestations had higher ESR and CRP values (Mann–Whitney U test, p  < 0.05 for all). Binomial logistic regressions showed that ESR had valuable predictive value for active BD (OR = 1.09 [1.04–1.13], AUC = 0.79 [0.74–0.83], p  < 0.001) and active vascular manifestations (1.03 [1.01–1.05], AUC = 0.85 [0.79–0.92], p  < 0.001). CRP had good predictive value for active vascular manifestations (OR 1.98 [1.45–2.72], AUC = 0.86 [0.8–0.91], p  < 0.001). The optimal value of ESR ≥ 10.5 and ESR ≥ 42.5 could predict active BD and active vascular manifestations with sensitivity, specificity = 71%, 75% and = 81%, 83% respectively. Conclusions ESR and CRP are both associated with active BD and most manifestations of the diseases. They can be used for the prediction of active BD and active vascular manifestations in BD patients. Further studies can help to confirm the findings of the current research.