Mortality of patients with bullous pemphigoid in Korea

Abstract

Background: The reported mortality rates and prognoses of bullous pemphigoid (BP) vary among different countries. However, the mortality rate of BP has not been investigated in Korea.

Objective: We sought to evaluate the mortality rate of Korean patients with BP in comparison to that of an age-matched general population in Korea, and to identify prognostic factors affecting overall survival.

Methods: We conducted a retrospective analysis of 168 patients diagnosed with BP between 1993 and 2013 at Gangnam Severance Hospital in Seoul, Korea.

Results: The 1-, 2-, and 5-year mortality rates of BP patients were 19.46% (95% confidence interval [CI], 13.83-27.00), 29.13% (95% CI, 21.91-38.08), and 58.03% (95% CI, 47.04-69.45), respectively. The median age was 73 years (range, 6-99 years). The standardized mortality ratio ranged from 2.43 (95% CI, 0.12-11.96) to 9.56 (95% CI, 2.43-26.02), depending on the age group. In multivariate analysis, old age at the time of diagnosis, stroke, diabetes, and delayed diagnosis were associated with increased mortality.

Limitations: This study was conducted as a retrospective analysis and was based at a single institution.

Conclusion: The mortality rate of patients with BP is significantly higher than that for the general population.

Keywords: Korea; autoimmune bullous disease; bullous pemphigoid; mortality; prognosis.