Unmasking Behçet's disease in the European population: a case series report.

Abstract

Behçet's disease (BD) is rare in European populations. Because clinical and immunological characteristics may differ between Caucasians and Middle Eastern patients, we analyzed BD patients from the Polish population. Data from eight patients with BD treated at a rheumatology clinic were analyzed and collected, including demographic data, clinical features, laboratory test results, comorbidities, and treatment options. The study included eight patients (mean age 38.2 ± 13.7 years; 75% female). None of the patients had a family history of the disease. The mean delay in diagnosis was 5.28 ± 4.6 years. The disease began with the development of mucosal ulcerations and a maculopapular rash, and some patients also developed anemia, alopecia, headaches, and arthritis. All patients had mucosal ulcerations and an erythematous or maculopapular rash; joint involvement occurred in 62.5%. Vascular complications (37.5%) included superior sagittal sinus thrombosis, ischemic stroke, and cephalic vein thrombosis. The most common comorbidities were hypertension (50%), dyslipidemia (37.5%), and depression (25%). Inflammatory markers were elevated in half of the patients and normalized after treatment. Antinuclear antibodies were positive in 62.5%, and antineutrophil cytoplasmic antibodies in 25%. Patients were treated with glucocorticosteroids (100%) in combination with azathioprine (62.5%), cyclosporine (25%), methotrexate (12.5%), and hydroxychloroquine (25%). Data from a single center indicate that BD in Caucasians is characterized by heterogeneity in clinical manifestations and develops predominantly in women. The presence of ANA and ANCA antibodiescan be misleading and requires differential diagnosis. The course is generally mild, but severe symptoms (especially thrombosis) require close monitoring by a multidisciplinary team.