Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population‐based cohort study*

Abstract

Summary Background A rising incidence and high mortality were found for bullous pemphigoid (BP) over a decade ago in the UK. Updated estimates of its epidemiology are required to understand the healthcare needs of an ageing population. Objectives To determine the incidence, prevalence and mortality rates of BP in England from 1998 to 2017. Methods We conducted a cohort study of longitudinal electronic health records using the Clinical Practice Research Datalink and linked Hospital Episode Statistics. Incidence was calculated per 100 000 person‐years and annual point prevalence per 100 000 people. Multivariate analysis was used to determine incidence rate ratios by sociodemographic factors. Mortality was examined in an age‐, sex‐ and practice‐matched cohort, using linked Office of National Statistics death records. Hazard ratios (HRs) were stratified by matched set. Results The incidence was 7·63 [95% confidence interval (CI) 7·35–7·93] per 100 000 person‐years and rose with increasing age, particularly for elderly men. The annual increase in incidence was 0·9% (95% CI 0·2–1·7). The prevalence almost doubled over the observation period, reaching 47·99 (95% CI 43·09–53·46) per 100 000 people and 141·24 (95% CI 125·55–158·87) per 100 000 people over the age of 60 years. The risk of all‐cause mortality was highest in the 2 years after diagnosis (HR 2·96; 95% CI 2·68–3·26) and remained raised thereafter (HR 1·54; 95% CI 1·36–1·74). Conclusions We report a modest increase in the incidence rate of BP, but show that the burden of disease in the elderly population is considerable. Mortality is high, particularly in the first 2 years after diagnosis.

Clinical characteristics, mortality, and prognostic factors for bullous pemphigoid in a Thai population

“…The median age of onset during the present study was 82 years, which was comparable with those reported in French and UK populations, which were 82 and 81 years, respectively. [ 4 – 5 ] However, most previous studies have reported a lower median age of onset, ranging from 70 to 80 years. [ 6 – 19 ] The previous study, from central Thailand, reported a median age of onset of 69 years, which was lower than that in the present study.…”

Assessment of the Characteristics and Associated Factors of Infectious Complications in Bullous Pemphigoid

“…It is an autoantibody-induced cutaneous inflammatory disease against BP180 or BP230 at the dermal-epidermal junctions (1,2). The annual incidence rate of BP has been increasing steadily in the elderly and general populations (3), and the BP patients were reported to have a significantly increased risk of death compared to the control subjects (4)(5)(6)(7). Conditions such as older age, poor general condition, dementia, comorbidities, and high-dose corticosteroids have been reported to be the predisposing factors for death (8)(9)(10)(11).…”

Autoantibodies Against the Immunodominant Bullous Pemphigoid Epitopes Are Rare in Patients With Dermatitis Herpetiformis and Coeliac Disease

“…BP typically affects people aged 75-80 years (1), while DH patients are generally younger, its incidence being highest in individuals aged 50-69 years (2). The incidence of BP has recently increased in several countries (3) and that of DH decreased (4). Recent epidemiological work revealed a special connection between these two autoimmune diseases: a prior diagnosis of DH was found to increase the risk of BP by 22-fold (5).…”