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Innate lymphoid cells (ILCs) are lymphoid cells that have important effector and regulatory functions in innate immunity and tissue remodeling. Uncontrolled activation and proliferation of ILCs can contribute to inflammatory autoimmune diseases. Behcet's disease (BD) is a complex systemic inflammatory disorder of unknown etiology. It has been shown that natural killer (NK) cells may play an immunoregulatory role in BD, however the role of ILCs is unknown. In this study, the levels and functions of ILCs and NK cell subsets in BD patients were investigated. Cell surface and cytotoxic granules (perforM and granzyme) expression of NK cells and ILCs were evaluated and labeled according to whole blood lysing protocol in peripheral blood samples obtained from the patients and healthy subjects. Cytokine levels of NK cells were investigated in stimulated peripheral blood mononuclear cells. All data were analyzed by flow cytometry. Total ILC and ILC3(+) cells were increased in active BD patients compared to inactive BD patients and healthy subjects. There was no significant difference between the patients and healthy subjects regarding NK cell surface and intracellular molecule expression. Although, an increase in IFN-gamma and IL-17, and a decrease in IL-4 levels were observed in CD56(dim) NK cell subset of BD patients. Recent studies showed increased neutrophilic infiltration and IL-17 secreting Th17 cells in BD patients. It is known that ILC3(+ )cells are similar to Th17 subset regarding their cytokine profile and transcription factor expression patterns. Results of current study may suggest that inflammatory microenvironment in BD patients might direct ILC cells to differentiate into ILC3(+) subset, and IL-17 released by NK cells might have a role in neutrophilic infiltration.

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.

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.

Background Vascular lesions can be the presenting sign of Behcet's disease (BD) preceding classical symptoms. The aim of this study was to evaluate the prevalence and types of vascular involvement in BD. Methods Among 2319 patients diagnosed with BD according to the criteria of the International Study Group for BD, 332 patients (279 male, 53 female) with vascular involvement were included in this study. Results Prevalence of vascular involvement was 14.3%. Vascular involvement was found to be more common in males (P

Background. Behcet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. Materials and Methods. Patients suffering from Behcet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behcet's disease (JBD), were detected among 1784 Turkish Behcet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 ± 7.1 years. Results. The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. Conclusions. Severe Behcet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.

Background The prevalence of Behcet'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.

Purpose: Behcet’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 (C3), 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 χ 2 Fisher exact test. Results: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C3 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C3. 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.