Serum Elafin as a Potential Marker of Disease Activity in Behçet's Disease.

Background: Elafin is a serine protease inhibitor with anti-inflammatory properties. It is expressed in various epithelial tissues with increased production under inflammatory conditions. Increased tissue elafin expression in Behçet's disease (BD) lesions has previously been demonstrated. Aims and Objectives: We hypothesised that serum elafin might be increased in patients with BD and aimed to assess the relationship of serum elafin with disease activity and organ involvement in BD. Materials and Methods: Fifty-four BD patients (29 active, 25 inactive) and 30 healthy controls were included in this single-centre case-control study. Serum elafin was measured using ELISA. Complete blood count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) tests were also performed. Results: Serum elafin levels were significantly higher in patients with BD (1.58 ± 0.47 ng/ml, range: 0.67-2.96) compared to controls (1.10 ± 0.28 ng/ml, range: 0.65-1.49) (P < 0.001). Patients with active BD had higher elafin levels than patients with inactive BD (P = 0.008). Active arthritis was associated with an increase in elafin (P = 0.012), while the presence of mucocutaneous symptoms was not. Serum elafin correlated significantly with ESR (P = 0.001). The ideal cut-off value for the diagnosis of BD was determined as 1.24 ng/ml with a sensitivity and specificity of 72.2% and 70.0%, respectively. Conclusion: Serum elafin is significantly increased in patients with BD. It may serve as a marker of disease activity, especially articular involvement.

The risk of endothelial and erectile dysfunctions in Behçet's disease: a comparative analysis of mucocutaneous and systemic patient groups.

INTRODUCTION: Behçet's disease (BD) is a chronic inflammatory disease which can be limited to only mucocutaneous tissues or can affect different systems of the body. AIM: To investigate the association of endothelial and erectile dysfunctions with BD, on the basis of comparative analysis between mucocutaneous and systemic BD. MATERIAL AND METHODS: Thirty-eight men diagnosed with BD were included in the present study. The patients were stratified into two groups as mucocutaneous BD (n = 20, MBD group), and systemic BD (n = 18, SBD group). Erectile dysfunction (ED) was assessed using the Erectile Function domain of the International Index of Erectile Function (IIEF-EF) questionnaire. The coronary flow reserve (CFR) assessment was done for analysing endothelial dysfunction (EnD), and CFR < 2 was defined as EnD. Penile Doppler ultrasonography (PDU) was performed for ED. The demographic and clinical parameters, IIEF-EF score ED classification, CFR and PDU test findings were compared between two groups. RESULTS: The median age was 34 (22-52) years in the overall population, and there was no difference between two groups (p = 0.558). Time from diagnosis was significantly longer (24 vs. 102 months, p = 0.021) and the use of immunosuppressive therapies was higher (0 vs. 70.6%, p < 0.001) in the SBD group. In overall, median CFR was 1.92 (1.1-5.96), and there was no difference between two groups (1.88 vs. 1.97, p = 0.812). The percentage of patients with CFR < 2 was similar in two groups (52.6% vs. 52.9%, p = 0.985). The ED status according to IIEF-EF was similar in two groups (45% vs. 27.8%, p = 0.538) as well as according to PDU analyses (10% vs. 16.7%, p = 0.544). CONCLUSIONS: The increased risk of endothelial, and erectile dysfunctions should be considered in men who were diagnosed with mucocutaneous and systemic BD.

Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease.

Behçet's disease is a genetically complex disease of unknown etiology characterized by recurrent inflammatory attacks affecting the orogenital mucosa, eyes and skin. We performed a genome-wide association study with 311,459 SNPs in 1,215 individuals with Behçet's disease (cases) and 1,278 healthy controls from Turkey. We confirmed the known association of Behçet's disease with HLA-B*51 and identified a second, independent association within the MHC Class I region. We also identified an association at IL10 (rs1518111, P = 1.88 x 10(-8)). Using a meta-analysis with an additional five cohorts from Turkey, the Middle East, Europe and Asia, comprising a total of 2,430 cases and 2,660 controls, we identified associations at IL10 (rs1518111, P = 3.54 x 10(-18), odds ratio = 1.45, 95% CI 1.34-1.58) and the IL23R-IL12RB2 locus (rs924080, P = 6.69 x 10(-9), OR = 1.28, 95% CI 1.18-1.39). The disease-associated IL10 variant (the rs1518111 A allele) was associated with diminished mRNA expression and low protein production.

Direct immunofluorescence in Behçet's disease: a controlled study with 108 cases.

PURPOSE: Behçet's disease (BD) is a disease of unknown etiology, which has multisystemic involvement. This multisystemic involvement might be the clue for an autoimmune pathogenesis. In order to evaluate an autoimmune pathogenesis, we examined immunoreactans depositions in the skin of BD patients. MATERIALS AND METHODS: The skin samples of 108 BD patients (28 perilesional skin, 44 positive pathergy test site, 22 negative pathergy test site, 14 normal skin) were examined for the depositions of immunoglobulin (Ig)M, IgG, IgA, complement 3 (C(3)), and fibrinogen (F) using direct immunofluorescence (DIF). The data were statistically compared to the DIF of 36 systemic lupus erythematosus (SLE) patients and 20 healthy controls using chi(2) Fisher exact test. RESULTS: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C(3) 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C(3). However, IgG deposition was higher in SLE while F deposition was higher in BD. In both BD and SLE, the Ig depositions were highly significant when the data were compared with the healthy controls. CONCLUSION: The significant deposition of immunoreactans in BD, especially in the negative pathergy and the normal skin sites, were observed. This study is the first controlled study revealing positive Ig depositions in BD, and it is expected to help us to reconsider the autoimmune pathogenesis in BD.

Prevalence of Behçet's disease in Istanbul, Turkey.

BACKGROUND: The prevalence of Behçet's disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Three previous epidemiologic surveys have been carried out in different regions of Turkey. PATIENTS AND METHODS: This study investigated the cross-sectional prevalence of BD in individuals aged > 12 years in Istanbul, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) by visiting them in their homes, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The sample size was determined to be 24,000 with an expected BD prevalence rate of 1/1000 and a sampling error of 4/10,000, with a 95% confidence interval (CI) of 6-14/10,000. The number of individuals to be screened in each district was determined in proportion to the population of all districts in Istanbul. RESULTS: The standard questionnaire was applied to a total of 23,986 individuals at their homes. A history of ROU was recorded in 2289 individuals (9.5%), and a previous diagnosis of BD was recorded in 47. The diagnosis of ROU was confirmed in 700, and the diagnosis of BD was established in 101 according to the International Study Group criteria. The prevalence rate of BD was estimated as 42/10,000 (95% CI, 34-51/10,000) in Istanbul, Turkey. CONCLUSIONS: This survey conducted in Istanbul, the largest cosmopolitan city in Turkey with immigrants from all over the country, has a larger sample size than other previous studies, and therefore the reported prevalence rate of BD has a more acceptable confidence interval. This study aids in the estimation of the prevalence of BD in Turkey, and supports previous findings that Turkey has the highest prevalence rate of the disease in the world.