Clinical course of behçet's disease patients followed-up in Behçet Disease Unit of Department of Dermatoveneorology of Akdeniz University Hospital

Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown aetiology. The natural course of BD is not fully known. Onset manifestation and time order or time intervals of the other symptoms varies from patient to patient. Factors affecting the clinical course and severity has not been clarified yet. In the present study, we aimed to determine the occurrence of the symptoms retrospectively in chronologic order. We also evaluated the influence of possible risc factors on the clinical severity of BD. A total of 368 patients with B.D. (171 female, 197 male; mean?SD age, 41,11±10,98 years), diagnosed according to the criteria of the International Study Group for Behçet's Disease were included in the study. The demographic findings and clinical features, retrospectively in chronologic order were recorded. Patients were also assessed for clinical severity score as previously described by Krause et al. Oral ulcers (OU) was the most common manifestation (100%) followed by genital ulcers (GU) (89.4%), papulopustular lesions (PPL) (75.0%), articular involvement (60.1%), erythema nodosum (EN) (44.3%) and ocular involvements (32.9%). Skin pathergy reaction was positive in 40,8% of cases, and HLA-B51 in 42,3% of cases examined. EN (p=0,031), and articular involvement (p=0,016) were more common in female patients, and eye involvement (p=0,023), thrombophelibitis (p=0,012), venous thrombosis (p=0,001) and neurologic involvement (p=0,023) in male patients. OU was the most common onset manifestation (66.8%) followed by GU (4.9%), EN (3.3%) and eye involvement (1.4%). The duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4,67±5,95 years. The duration between the time point of fulfilment of diagnostic criteria and the diagnosis (2,5±2,11) was found to be longer in patients having only mucocutaneous lesions (1,86±1,58 years) than in patients having serious organ involvement (1,86±1,58 years) (p=0,001). Mean clinical severity score (5,07±1,94) was higher in male patients (5,31±2,09) compared with female patients (4,78±1,72) (p=0,015). The clinical severity score increased significantly with the passage of time (p= 0,0001, r=0,223). In logistic regression analysis, male gender (p=0.03) and increased numbers of symptoms at the diagnosis (p<0.0001, R2=0,852) were found to be a significant risk factors for the severity of B.D. Our results indicate that mucocutaneous lesions are hallmarks of the disease, and especially OU precede other manifestations. Male sex and patients with increased numbers of organ involvement at the diagnosis are associated with more severe disease. Therefore, close monitoring and appropriate treatments are mandatory to decrease the morbidity and mortality of the disease in these patients