Background Behcet’s disease (BD) is a multisystem disease with vasculitic features and possibly an autoinflammatory pathogenesis. It is rare in Germany with an estimated prevalence of 0,6/100.000 inhabitants, whereas it is much more common in Turkey where, depending on the region, its prevalence ranges from 40 to 340/100.000.
Objectives To evaluate the relationship between ethnic origin, disease manifestations and the association of HLA-B 51 to the disease itself and its expression in German and Turkish patients living in Germany.
Methods 238 patients with BD regularly seen in a large interdisciplinary reference center for Behçet’s disesase were retrospectively analysed. 112 were of German and 126 of Turkish origin, all living in Germany. They all fulfilled the criteria of the International Study Group of BD. The entire spectrum of disease was analysed, all patients were seen by a rheumatologist and ophthalmologist and a laboratory workup including HLA-B51 genotyping was done. Disease severity was scored according to an established scoring system. Fisher’s Exact test and Chi Square test as well as Kaplan Meier curves and Odds ratios were calculated.
Results Males are more commonly affected than females (3:1) in the Turkish versus the German patient group (1:1), OR 2,62. In German patients, time from first manifestation to diagnosis was 4 years versus one year in Turks (p<0,0015). All disease manifestations and disease severity were comparable in both patient groups. HLA B51 was positive in 36% of the German and 77% of Turkish patients (p<0,001). Ocular involvement was significantly associated with HLA-B 51 in both patient groups, in German patients (p<0,007) even more than in Turkish patients (p<0,05). Moreover, in Turkish patients, a significant association between papulopustules and HLA-B 51 was found, this was not the case in German patients.
Conclusions In general, BD is very similar in patients of Turkish and German origin living in Germany. There are some similarities between Turks living in Germany and Turks living in Turkey: the gender difference is identical, as is the high association with HLA-B51. However, the frequency of pathergy positivity is lower in Turks in Germany than in those in Turkey (17% vs. 30%) and in this respect identical with that of German BD patients. The first finding underlines the contribution of genetics, whereas the latter probably is due to environmental influences. Pathergy positivity is reported to be much rarer in Northern European BD patients than in Middle Eastern patients. In order to further evaluate these findings, a direct comparison of the German patient cohort with one of Turkish patients living in Turkey using the same methods as in the present study would be valuable.
Kotter, I., R. Vonthein, et al. (2004). Behcet’s disease in patients of German and Turkish origin living in Germany: a comparative analysis. J Rheumatol 31(1): 133-139.
Krause, L., A. K. Kohler, et al. (2009). Ocular involvement in Adamantiades-Behcet’s disease in Berlin, Germany. Graefes Arch Clin Exp Ophthalmol 247(5): 661-666.
Disclosure of Interest None Declared
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