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

Persson, M. S.M.; Harman, K. E.; Vinogradova, Y.; Langan, S. M.; Thomas, K. S.; Hippisley-Cox, J.; Gran, S.

Abstract

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 100000 person-years and annual point prevalence per 100000 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 100000 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 100000 people and 141·24 (95% CI 125·55–158·87) per 100000 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.

Citation

Persson, M. S., Harman, K. E., Vinogradova, Y., Langan, S. M., Thomas, K. S., Hippisley-Cox, J., & Gran, S. (2021). Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population-based cohort study*. British Journal of Dermatology, 184(1), 68-77. https://doi.org/10.1111/bjd.19022

• Journal Article Type: Article
• Acceptance Date: Mar 5, 2020
• Online Publication Date: Mar 9, 2020
• Publication Date: Jan 1, 2021
• Deposit Date: Mar 10, 2020
• Publicly Available Date: Mar 10, 2021
• Journal: British Journal of Dermatology
• Print ISSN: 0007-0963
• Electronic ISSN: 1365-2133
• Publisher: Wiley
• Peer Reviewed: Peer Reviewed
• Volume: 184
• Issue: 1
• Pages: 68-77
• DOI: https://doi.org/10.1111/bjd.19022
• Keywords: Dermatology
• Public URL: https://nottingham-repository.worktribe.com/output/4121532
• Publisher URL: https://onlinelibrary.wiley.com/doi/10.1111/bjd.19022
• Additional Information: This is the peer reviewed version of the following article: Persson, M., Harman, K., Vinogradova, Y., Langan, S., Hippisley?Cox, J., Thomas, K. and Gran, S. (2020), Incidence, prevalence, and mortality of bullous pemphigoid in England 1998?2017: a population?based cohort study. Br J Dermatol., which has been published in final form at https://doi.org/10.1111/bjd.19022. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions.