Document Type : Original Article

Authors

• Fatemeh Afagh Abedi Mamaghani ¹
• Farhad Shahram ²
• Fereydoun Davatchi ³
• Christos C Zouboulis ⁴

¹ DGP- Deutsche Gesellschaft fuer Pneumologie Aerztekammer Nordrhein

² Professor of Rheumatology, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran

³ Rheumatology Research Ctr Shariati Hospital-Tehran Univ Jalal AI-Ahmad Avenue Tehran 14117 IRAN

⁴ German Registry of Adamantiades-Behçet's Disease, Department of Dermatology, University Medical Center Benjamin Franklin, Free University of Berlin, Germany

Abstract

Adamantiades-Behçet’s disease (ABD) is a chronic, recurrent inflammatory disease with an unclear aetiology. Major and minor clinical signs characterize ABD. Major signs generally comprise oral and genital aphthae and skin and eye manifestations. Minor signs include joint, neurological, and gastrointestinal symptoms and vascular involvement. This work aims to compare the prevalence and clinical manifestations of ABD using available study results from Iran and Germany.This report is based on statistical data from Iran and Germany. A long-term study over 35 years from the Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, and a large German epidemiological study dealing with data from the German Registry for ABD and data on the disease in Europe are the information sources. Additionally, data from recent ABD studies listed in PubMed are used.Although the prevalence of ABD in Iran, with 80 patients per 100,000 inhabitants, is markedly higher than in Germany, with 4.2 patients per 100,000 inhabitants, the male-to-female ratio and age of onset are similar in the two populations. In Iran, the male-to-female ratio is 1.3:1, and the average onset age is 26 ± 11.3 years. In Germany, the male-to-female ratio is 1.4:1, and the average onset age is 27.7 ± 11.6 years. The incidence of the disease is decreasing, both in Iran and other parts of the world.Recurrent oral aphthae were the most common onset manifestation in patients of both Iran (82.7%) and Germany (84.5%), with ocular manifestations more common onset signs in Iran (8.7%) than in Germany (5.1%). Except for recurrent genital ulcers, which were more common in women in both countries, all other clinical signs – except oral aphthae – were more common in male patients in both countries. Minor clinical signs differed.A markedly different prevalence was observed in the study populations of Iran and Germany, with the disease being more common in Iran. However, despite the different origins, demographics and major clinical signs of ABD were similar in both countries. ABD is most frequently detected in young patients.

Keywords

• Adamantiades-Behçet’s disease
• epidemiology
• clinical signs, Iran
• Germany

Main Subjects

• Behest's Disease