Khalaf Kridin, Wesal Shihade, Reuven Bergman
DOI: 10.2340/00015555-2930

There is little consensus regarding mortality data in bullous pemphigoid (BP). The aim of this study was to evaluate mortality among a relatively large cohort of Israeli patients with BP and to perform a meta-analysis synthesizing existing data on 1-year mortality rates of patients with BP. This retrospective cohort study of 287 patients diagnosed with BP between 2000 and 2015 compared the mortality of patients with BP with age- and sex-matched control subjects in the general population. The results showed 1-, 5- and 10-year mortality rates of 26.9%, 56.9% and 69.5%, respectively, and a 3.4-fold higher risk of death. A systematic review and meta-analysis were then performed using a random effects model. Including the current study, 25 studies comprising 4,594 patients met the eligibility criteria. The pooled estimate of 1-year mortality rate was 23.5% (95% confidence interval 20.2–26.8; I2=81%; p < 0.001). The pooled 1-year mortality rate of European cohorts was prominently higher relative to the pooled rates of cohorts from the USA and Asia.

Mortality data in BP are highly inconsistent. By this retrospective cohort study, a 3.4-fold increased mortality and 27% 1-year mortality rate were recognized among Israeli patients with BP. Performing a systematic review and meta-analysis led to the conclusion that the pooled estimate of 1-year mortality rate in BP patients is 23.5% worldwide. The stratified pooled estimate is higher in European cohorts as compared to American and Asian ones. These data allow patients to be aware of prognosis and to make informed decisions, and provide clinicians with an indicator of the success of treatment.