Behcet's Disease in Iran, Analysis of 5,059 Cases

BACKGROUND—Iran is among the countries with rather high prevalence of Behcet''s disease (BD). We present here our latest data on different aspects of the disease. Materials AND METHODS—The present investigation is a prospective cohort study carried out on the data of patients presented in our BD registry during the past 28 years. The data were collected on a standard protocol comprising 100 items. These items included demographic features (such as sex, age of onset, age of diagnosis, date of the first visit, and ethnic origin), type of the presentation, different clinical manifestations of the disease, and paraclinical findings (including CBC and platelet count, Erythrocyte sedimentation rate, VDRL/RPR test, urinalysis, HLA typing, and Pathergy skin test). A confidence interval (CI) at 95% was calculated for each item. RESULTS—A total number of 5,059 patients were analyzed. The annual incidence rate was 280 patients in the last 5 years. The male/female ratio was 1.19/1 and the mean age at onset was 26 ± 9.8 (CI: 0.3). As the first manifestations, oral aphthosis (OA) was the most frequent (81%, CI: 1.1). The prevalence of variuos manifestations were OA: 97% (CI: 0.5), genital aphthosis: 65% (CI: 1.3), skin: 69% (CI: 1.3), ocular: 56% (CI: 1.4), joint: 34% (CI: 1.3), CNS: 3% (CI: 0.5), vascular: 8.5% (CI: 0.8), GI: 8% (CI: 0.8), and epididymitis: 10% (CI: 1.1). The laboratory findings were as follows: high ESR: 53% (CI: 1.4), urine abnormality: 10% (CI: 0.9), positive pathergy test: 57% (CI: 1.4), HLA B5: 52.5% (CI: 1.4), HLA B51: 34% (CI: 5.1), and HLA B27: 9% (CI: 0.8).CONCLUSION—Recent survey in Iran revealed a remarkable decrease of the incidence rate of BD and a tendency toward milder forms of the disease. Our data show more similarity with those of Turkey and Japan than with the western parts of the world.