초록/요약

Background: Studies investigating the epidemiology and mortality of Takayasu Arteritis (TA) are scarce. The objective of this study was to provide information regarding the epidemiology of TA, such as the incidence, prevalence, survival and cause of death in Korea. Methods: We used a national, population-based database maintained by the Rare Intractable Disease registration program and the Health Insurance Review and Assessment Service, which includes information on all TA patients, diagnosed based on uniform criteria. We also used data from Statistics Korea linked with RID database to confirm survival and cause of death. We analyzed data for the period 2008-2012. Results: During study period, there were 612 new patients with TA, with a female-to-male ratio of 4.3:1. The annual mean incidence was 0.24 per 105 people, and the prevalence was 2.82 per 105 people in 2012. The average annual mortality rate was 0.02 per 105 people, and the standardized mortality ratio was 3.1 per 105 people. The overall survival of patientswith TA was 96.6%, whichwas significantly lower than that in the general population. Cardiovascular disease was the most common cause of death, accounting for 29 of the 64 deaths (45.3%), followed by neoplasms in 9 patients (14.1%). Conclusions: The incidence and prevalence presented in this study are the highest ever reported. The survival of patients with TA was significantly lower than that of the general population, and the main cause of death was cardiovascular disease. This study reflects the need for greater awareness of TA, especially in Asian countries. (C) 2017 Elsevier B.V. All rights reserved.