Behçet disease: a rare systemic vasculitis in Poland.

INTRODUCTION    Behçet disease (BD) is an immune‑mediated small‑vessel systemic vasculitis, which is rarely seen in Poland. OBJECTIVES    The aim of this study was to evaluate the incidence and prevalence of BD, as well as to assess the sex and geographical distribution of BD in Poland during a 7‑year follow-up. To our knowledge, this is the first evaluation of this rare disease in Poland, based on a hospital morbidity database. PATIENTS AND METHODS    We analyzed population‑based administrative data obtained from a national hospital morbidity study conducted between January 2008 and December 2014 by the National Institute of Public Health in Poland. The annual incidence rates and point prevalence of BD were calculated using the number of patients with BD and corresponding census data for the overall Polish population.  RESULTS    Data included 316 hospitalization records. The final study sample comprised 130 patients (54 men [42%] and 76 women [58%]) with first‑time hospitalizations for BD. The mean (SD) age was 41.6 (18.7) years (95% confidence interval [CI], 38.3-44.8; range, 5-85 years). The incidence rate of BD was estimated at 0.5 per million persons per year (95% CI, 0.35-0.61). The point prevalence at the end of 2014 was 3.4 per million persons. The incidence rate of BD did not vary significantly between more urban and more rural regions, and BD was observed more often in women than in men. CONCLUSIONS    BD is endemic in Eastern and Central Asian countries, but is also seen in Poland. However, its incidence and prevalence rates are lower in Poland than in other European countries.