Characteristics of late-onset Behçet's disease and comparison with juvenile and adult-onset Behçet's disease.

BACKGROUND: The course of late-onset Behçet's disease (LBD) is unknown. This study aimed to determine the characteristics and systemic involvement of LBD. METHODS: In this retrospective, cross-sectional, and comparative study, 700 patient files from 4100 patients diagnosed with Behçet's disease (BD) were selected and divided into three groups: LBD, adult BD (ABD), and juvenile BD (JBD). The age limits for LBD and JBD were determined to be 42 and 16, respectively. LBD patients were compared to ABD and JBD patients in terms of demographics, systemic involvement, and disease duration. RESULTS: The LBD rate among BD patients was 4.7% (183/4,100). The time from initial symptom occurrence to the age at which the BD criteria were met was longer in LBD than in ABD or JBD. Except for genital ulcers, the frequencies of involvement in LBD were similar to those in ABD and JBD. The frequency of family history was significantly lower in LBD (12%) than in ABD (15.9%) or JBD (18.2%). The time from the initial symptom to oral aphthae, genital ulcers, and eye involvement was longer in LBD than in ABD or JBD. Furthermore, erythema nodosum was observed after a longer duration in LBD than in ABD. CONCLUSION: Considering that involvement occurs much later in LBD and there are no differences in the frequencies of involvement except for genital ulcers, LBD patients should be followed up as closely as ABD and JBD patients.

Inflammatory status might direct ILC and NK cells to IL-17 expressing ILC3 and NK subsets in Behcet's disease.

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 (perforin 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-γ and IL-17, and a decrease in IL-4 levels were observed in CD56dim 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.

Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases.

BACKGROUND: Vascular lesions can be the presenting sign of Behçet'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<0.001, male:female ratio 5.26:1). Mean age at onset of vascular involvement was 30.58+/-7.88. Vascular lesion was the presenting sign of BD in 50 patients (2.1%), whereas in 109 patients (4.7%) it started together with other clinical symptoms at the age of diagnosis. Superficial vein thrombophlebitis (SVT) was the most common vascular symptom (53.3%) followed by deep vein thrombosis (DVT) (29.8%). Arterial lesions were rare (3.6%). Of the patients 13.3% had more than one type of vascular involvement. Lower extremities were the most frequent localization in patients with SVT, whereas the femoral vein was mainly involved in patients with DVT and the pulmonary artery was mainly involved in the patients with arterial lesions. CONCLUSION: In countries like Turkey where BD is more frequent, young male patients presenting with vascular involvement should be evaluated for BD.