Cause-specific mortality in people with bullous pemphigoid and pemphigus vulgaris: a systematic review and meta-analysis

Hasanaj, Arbi; Zaki, Faaris; Harman, Karen E.; Grindlay, Douglas; Gran, S.

Abstract

Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are autoimmune blistering skin disorders which are increasing in incidence and are associated with high mortality. 1,2 As people with BP and PV often have comorbidity and require treatment that has severe side-effects, they could die of various causes. 3,4,5 A systematic review on cause-specific mortality in has not previously been conducted. The aim of this study was to examine causes of death in patients with BP and PV. The study protocol was registered with PROSPERO (CRD42020202620); PRISMA guidelines were followed. On 10 th October 2020, a search was conducted in MEDLINE, Embase and Cochrane Central Register of Controlled Trials. Grey literature was identified using the EthOs library and OpenGrey. Randomised control trials (RCTS) and cohort studies were included due to follow-up being integrated within the study designs. To be included, studies had to report at least one measure of cause-specific mortality in people with BP or PV. All languages were included. Three authors performed independent study screening, data extraction and quality assessment, using the Joanna Briggs Institute tool for cohort studies. 6 Disparities were resolved by discussion. We defined high-quality studies as those with at least one-year of follow-up and a control group matched for age and gender as these criteria minimise bias and confounding. For each study, the proportional mortality (PM) was calculated by dividing the number of deaths from a specific cause by the total number of deaths. The standardised mortality ratio (SMR) was calculated by observed deaths divided by expected deaths of the reference population. Meta-analyses were conducted using a Der Simonian-Lairds random-effects model, determining (i) the pooled PM and, (ii) the pooled SMR for causes of death where available. 7 Subgroup analysis was not carried out due to insufficient data. Heterogeneity was assessed using I 2 (>75% defined as considerable heterogeneity).

Citation

Hasanaj, A., Zaki, F., Harman, K. E., Grindlay, D., & Gran, S. (2022). Cause-specific mortality in people with bullous pemphigoid and pemphigus vulgaris: a systematic review and meta-analysis. British Journal of Dermatology, 186(2), 359-361. https://doi.org/10.1111/bjd.20759

• Journal Article Type: Letter
• Acceptance Date: Sep 8, 2021
• Online Publication Date: Nov 15, 2021
• Publication Date: Feb 1, 2022
• Deposit Date: Sep 28, 2021
• Publicly Available Date: Nov 16, 2022
• Journal: British Journal of Dermatology
• Print ISSN: 0007-0963
• Electronic ISSN: 1365-2133
• Publisher: Oxford University Press (OUP)
• Peer Reviewed: Peer Reviewed
• Volume: 186
• Issue: 2
• Pages: 359-361
• DOI: https://doi.org/10.1111/bjd.20759
• Public URL: https://nottingham-repository.worktribe.com/output/6344180
• Publisher URL: https://onlinelibrary.wiley.com/doi/10.1111/bjd.20759
• Additional Information: This is the peer reviewed version of the following article: Hasanaj, A., Zaki, F., Harman, K., Grindlay, D. and Gran, S. (2022), Cause-specific mortality in people with bullous pemphigoid and pemphigus vulgaris: a systematic review and meta-analysis. Br J Dermatol, 186: 359-361, which has been published in final form at https://onlinelibrary.wiley.com/doi/10.1111/bjd.20759. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited