Department of Dermatology, Peking University First Hospital, Beijing, China,
National Clinical Research Center for Skin and Immune Diseases, Beijing, China,
Dermatology. 2023;239(4):514-522. doi: 10.1159/000530121. Epub 2023 Mar 21.
Pemphigus vulgaris is a life-threatening autoimmune bullous disease characterized by flaccid blister formation. As there has been no macroscopic assessment of epidemiological characteristics, its disease burden in the general population remains unknown.
The aim of this study was to assess the global incidence rate of pemphigus vulgaris in the general population.
The search was conducted in databases including Medline, Embase, Web of Science, and the Cochrane Library from inception to May 1, 2022. We included original studies that either reported the incidence of pemphigus vulgaris or provided raw data for calculating. Studies based on a specific population instead of the general population were excluded. Individual studies were summarized using random-effects mode. The pooled incidence rate of pemphigus vulgaris among the general population and subgroups was obtained. Heterogeneity (I2 statistic) was assessed with the χ2 test on Cochrane's Q statistic.
Twenty-nine studies were eligible for final analysis, and the pooled incidence rate of pemphigus vulgaris was 2.83 per million person-years (95% CI, 2.14-3.61). The incidence rate was similar between men and women and remained stable in the past half-century. Southern Asia showed the highest rate among subcontinents that had more than one study conducted as 4.94 per million person-years (95% CI, 2.55-8.10). Economic levels do not seem to have any bearing on incidence.
Despite the substantial heterogeneity among studies, this meta-analysis revealed the worldwide incidence rate of pemphigus vulgaris for the first time and may assist in assessing global disease burden and promoting health policy.
寻常型天疱疮是一种危及生命的自身免疫性大疱性疾病，其特征为松弛性水疱形成。由于缺乏对流行病学特征的宏观评估，其在普通人群中的疾病负担尚不清楚。
本研究旨在评估寻常型天疱疮在普通人群中的全球发病率。
检索数据库包括 Medline、Embase、Web of Science 和 Cochrane Library，检索时间截至 2022 年 5 月 1 日。我们纳入了报告寻常型天疱疮发病率或提供发病率原始数据的原始研究。排除仅针对特定人群而非普通人群的研究。使用随机效应模型对个体研究进行总结。获得普通人群和亚组中寻常型天疱疮的总发病率。采用 Cochrane's Q 统计量的 χ2 检验评估异质性（I2 统计量）。
共有 29 项研究符合最终分析条件，寻常型天疱疮的总发病率为 2.83/100000 人年（95%CI，2.14-3.61）。男性和女性的发病率相似，在过去半个世纪中保持稳定。与其他有一个以上研究的次大陆相比，南亚的发病率最高，为 4.94/100000 人年（95%CI，2.55-8.10）。经济水平似乎与发病率无关。
尽管研究间存在很大的异质性，但这项荟萃分析首次揭示了寻常型天疱疮的全球发病率，这可能有助于评估全球疾病负担并促进卫生政策。
