Harald Gollnick

Adamantiades-Behçet’s Disease: Epidemiology in Germany and in Europe

The German Registry of Adamantiades-Behcet’s disease founded 1990 in Berlin provides current data on the epidemiology, the clinical manifestations and the course of the disease in Germany on a continuous basis. A total number of 265 patients, including 106 German and 119 Turkish patients, has been reported to the German Registry until December 1998. Two hundred thirty-four patients fulfilled the criteria of the Behcet’s Disease Classification Tree. The prevalence of the disease evaluated in Berlin-West was 1.68/100,000 in 1989 and had risen to 2.26/100,000 in 1994. The median age of onset was 25 years (range 4 months to 72 years; German-Turks, ns). Juvenile disease was recorded in 6.3% of the patients. The complete clinical picture according to the criteria of the International Study Group of Behcet’s disease developed in 13 months. The interval between onset of the disease and diagnosis was 32 months, which was significantly longer than the duration of the development of the complete clinical picture (p<0.0001). The disease was diagnosed later in German (47 months) than in Turkish patients (23 months, p=0.001). While German patients presented an equal male-to-female ratio, a male predominance was shown in Turkish patients (M:F 2.1:1, p=0.013). Familial occurrence was detected in 3.2% of German and 15.5% of Turkish patients (p=0.016). The frequencies of major clinical manifestations were: oral ulcers 99%, skin lesions 75%, genital ulcers 74%, ocular manifestations 57%, arthritis 56%, and positive pathergy test 45%. Clinical differences between German and Turkish patients were found in the frequency of ocular lesions (47% vs. 63%, p=0.031), genital ulcers (82% vs. 69%, p=0.033) and prostatitis/epididymitis (26% vs. 11%, p=0.047). Oral ulcers were with 75% the most common onset sign of the disease followed by erythema nodosum (7%), arthritis (6%), uveitis (5%), genital ulcers (4%), superficial thrombophlebitis (1%) and papules/sterile pustules (1%). There was a trend for uveitis and erythema nodosum as onset signs to shorten the median interval to diagnosis to 1.5 and 15 months, respectively, and for arthritis to delay diagnosis (43.5 months; p=0.068). A severe course developed in 23% of the patients; irreversible retinal vasculitis to blindness in 13%, sterile meningoencephalitis in 6%, severe arthritis in 4%, hemoptysis in 2%, lethal outcome in 2% and bowel perforation in 1%. All 4 patients with lethal outcome were of German origin (4.2%). The relative risk of HLA-B51-positive German natives to develop the disease was 6.0 for males (p<0.001), while females showed no association of HLA-B51 with the disease. HLA-B51 was confirmed as marker of severe prognosis. Cardiolipin autoantibodies were associated with cutaneous vasculitis and neurological manifestations, and superficial thrombophlebitis was correlated with systemic vessel involvement.