A retrospective clinical analysis of korean patients with bullous pemphigoid

Abstract

Background : Bullous pemphigoid (BP) is a potentially fatal autoimmune bullous disease which commonly occurs in elderly patients. The mortality rate and prognosis of BP have been controversial in the populations of different countries. However, there are few studies to date that have investigated BP in Korea.Objective : To evaluate the mortality rates of Korean patients with BP and compare them with age-matched general population in Korea, and to identify prognostic factors associated with overall survival.Methods : We conducted a retrospective analysis of 168 patients diagnosed with BP between 1993 and 2013 in Gangnam Severance Hospital. The mortality rates of patients with BP were compared with that of age-matched general Korean population.Results : The 1-, 2-, and 5-year mortality rates of BP patients were 19.46%, 29.13%, and 58.03%, respectively. The standardized mortality ratio (SMR) varied from 2.43 to 9.56, depending on the age group. Sex, age at the time of diagnosis, stroke, cancer, and Karnofsky performance status score were associated with decreased overall survival on univariate Cox regression analysis. However, only age at the time of diagnosis and stroke were associated with increased mortality on multivariate analysis.Conclusions : The mortality rates of patients with BP are significantly higher than those of the general population in Korea. Advanced age at the time of diagnosis and stroke are strong prognostic factors associated with increased mortality.