Mortality in bullous pemphigoid: A systematic review and meta‐analysis of standardized mortality ratios

Abstract

There are inconsistent data on mortality rates in patients with bullous pemphigoid (BP). Trends in mortality in BP throughout the years are yet to be established. The aim of the present study was to study the mortality in BP patients relative to the general population and to estimate trends in standardized mortality over the past 30 years. We performed a systematic review and meta-analysis of observational studies in Medline, Embase and Scopus (1823-2017). Reference lists of included studies were also searched for eligible studies. Quality of evidence was assessed using the Newcastle-Ottawa Scale (NOS). A meta-analysis was performed using random-effects models to estimate pooled standardized mortality ratios (SMR) with 95% confidence intervals (CI). Meta-regression models were used to investigate the secular trends in SMR. Ten studies were included covering the period 1960-2015 (1736 patients, 746 deaths). Pooled all-cause SMR was 3.6 (95% CI, 2.6-5.0). There was no trend in all-cause SMR across the last three decades (regression coefficient 0.02 [change in logSMR/year]; 95% CI, 0.04-0.08; P = 0.545). In conclusion, there is a 3.6-fold increased mortality among patients with BP as compared with the age-matched general population. The excess mortality in BP has not changed significantly over the past 30 years.

Dual inhibition of complement factor 5 and leukotriene B4 synergistically suppresses murine pemphigoid disease

“…PDs are associated with an increase in mortality. Bullous pemphigoid (BP), for example, is associated with a 3.6-fold increase in mortality in the first year after diagnosis (2,3). The cause for the increased mortality is poorly understood, but adverse effects of immunosuppressants, in particular steroids, are believed to contribute substantially (2), illustrating the need for more selective therapeutic agents associated with fewer side effects.…”

The Bidirectional Association Between Bullous Pemphigoid and Psoriasis: A Population-Based Cohort Study

“…Bullous pemphigoid (BP) is the most prevalent subepidermal autoimmune blistering disease (AIBD) worldwide ( 1 ). Recent evidence accumulated to suggest that BP confers an increasing inpatient burden ( 2 ) owing to its growing incidence ( 3 ), its increased associated mortality ( 4 , 5 ), and high healthcare costs ( 6 ). This tissue-specific autoimmune disease arises from the production of IgG autoantibodies targeting the hemidesmosomal proteins BP180 and BP230 ( 7 ) and typically afflicts the elderly population ( 1 ).…”

Validation study of bullous pemphigoid and pemphigus vulgaris recording in routinely collected electronic primary healthcare records in England

“…Bullous pemphigoid and pemphigus vulgaris are rare autoimmune blistering diseases characterised by blistering of the skin and mucous membrane and associated with high mortality. 1 2 There is a continued need to understand the causes, natural history and disease associations in order to better inform patients and plan treatment. However, there is a scarcity of recent epidemiological research on these distinct diseases in the UK and many unanswered questions.…”