The natural course and factors affecting severity of Beh‡et's disease: a single-center cohort of 368 patients

Our goal was to determine, retrospectively, the occurrence of the symptoms of Beh double dagger et's disease in chronological order and the course of the disease. Additionally, probable factors affecting the clinical severity were investigated. A total of 368 patients (171 females and 197 males; aged 41.11 +/- A 10.9 years) were included in this retrospective cohort study. The chronological order of the clinical manifestations was recorded. Patients were also assessed for clinical severity score. Oral ulcer was the most common manifestation (100 %) followed by genital ulcer (89.4 %), papulopustular lesions (75 %) and articular involvement (60.1 %). Oral ulcer was the most common onset manifestation (66.8 %) followed by genital ulcer (4.9 %), erythema nodosum (3.3 %) and ocular involvement (1.4 %). The duration between the onset symptom and the fulfillment of the diagnostic criteria was 4.67 +/- A 5.9 years. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.5 +/- A 2.1 years) was longer in patients having only mucocutaneous lesions (2.8 +/- A 2.2 years) than in patients having serious organ involvements (1.9 +/- A 1.6 years; p < 0.01). Serious involvements such as neurological involvement and large vessel involvement had their onsets later. Mean clinical severity score was higher in male patients (5.3 +/- A 2.1 vs 4.8 +/- A 1.7; p < 0.05). In logistic regression analysis, male gender (p = 0.03) and increased number of symptoms at diagnosis (p < 0.001, R (2) = 0.73) were found to be significant risk factors for severity. Mucocutaneous lesions, especially oral and/or genital ulcers, usually precede possible serious involvements; therefore, careful follow-up is mandatory. Males with increased number of organ involvements at the diagnosis are associated with more severe disease.