Epidemiological features of Adamantiades-BehÇet's disease in Germany and in Europe

Abstract

The German Registry of Adamantiades-Behçet's disease was founded in 1990 in Berlin and it provides current data on the epidemiology, the clinical manifestations and the course of the disease in Germany on a continuous basis. A total of 218 patients, including 89 German and 100 Turkish patients, had been reported to the German Registry until October 1997. One hundred and ninety-six patients fulfilled the criteria of the Behçet'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 by 1994. The median age of onset was 25 years (range 5 to 66 years; German-Turks, ns). Juvenile disease was recorded in 6.9% of patients. The complete clinical picture according to the criteria of the International Study Group of Behçet's Disease developed in 15.5 months. The interval between onset of the disease and diagnosis was 35 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 (48.5 months) than in Turkish patients (25.5 months, p = 0.003). While German patients presented an equal male-to-female ratio, a male predominance was shown in Turkish patients (M:F 2.1:1, p = 0.022). Familial occurrence was detected in 2.0% of German and 15.9% of Turkish patients (p = 0.013). The frequencies of major clinical manifestations were: oral ulcers 99%, skin lesions 76%, genital ulcers 75%, ocular manifestations 59%, arthritis 59%, and positive pathergy test 52%. Clinical differences between German and Turkish patients were only found in the frequency of ocular lesions (48% vs. 66%, p = 0.025). Oral ulcers were with 72% the most common onset symptom of the disease followed by erythema nodosum (9%), uveitis (7%), arthritis (7%), genital ulcers (3%), superficial thrombophlebitis (2%) and papules/sterile pustules (2%). Uveitis and erythema nodosum as onset symptoms shortened the median interval to diagnosis to 1.5 and 15 months, respectively, while arthritis delayed diagnosis (43.5 months; p = 0.029). A severe course developed in 25% of the patients; irreversible retinal vasculitis to blindness in 15%, sterile meningoencephalitis in 8%, severe arthritis in 5%, hemoptysis in 2%, lethal outcome in 2% and bowel perforation in 1%. The relative risk of HLA-B5 positive German natives developing the disease. HLA-B5 was confirmed as a marker of severe prognosis. Cardiolipin autoantibodies were associated with cutaneous vasculitis and superficial thrombophlebitis was correlated with systemic vessel involvement.

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

“…Consistent with previous data, we found an annual incidence rate of 0.24 per 100,000, which is lower than that observed in Iran (29), Spain (30), and Greece (31), where the annual incidence rates are 0.58 per 100,000, 0.66 per 100,000, and 4 per 100,000, respectively. With regard to the clinical features of BD, in our population the age at diagnosis and the frequency of the specific clinical manifestations were similar to those observed in other European countries and in a previous hospital-based multicenter Italian study (6,32). In particular, we did not observe a significant preponderance of men, and the onset of BD at Ͻ16 years of age was exceptional.…”

“…We observed an 11% frequency of positive pathergy tests, which is similar to that reported in Spanish patients (30), but is much lower than that reported in Middle Eastern countries and Japan (21,(33)(34)(35). The frequencies of ocular lesions and neuroBehçet's (55.6% and 11.1%, respectively) were similar to those reported in other European countries (32). Male patients had posterior eye disease (77.8%) more often than female patients (22.2%).…”

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

“…The prevalence of familial ABD is in the range of 1-18%. 8,9,[13][14][15][16][17][18][19] In a study from Turkey, the risk of disease in sisters was 11.4-52.5. 19 Among different genetic markers, the class I HLA-B5 and -B51 gene on chromosome 6p21 is in association with ABD.…”

“…In addition, several studies have shown an association between HLA-B51 and ABD. [9][10][11] So far, the role of HLA-B51 regarding the incidence of this disease or its expression is not completely understood. It was shown that HLA-B5-positive individuals from northern Europe have a lower risk to develop the disease compared with patients from the southern parts of Europe.…”

One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet’s disease refractory to standard immunosuppressive drugs

“…Repeated attacks of uveitis can cause blindness [1][2][3]. Involvement of the gastrointestinal tract, central nervous system, and large vessels is less frequent, although it can be life threatening [1,2,[4][5][6].…”