中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (4): 254-260.doi: 10.12144/zgmfskin202604254

• 论著 • 上一篇    下一篇

基于贝叶斯模型的天疱疮全球发病率与疾病负担估算

李洋1,2,3,王真真2,3,解蓝宁1,2,3,李文超2,3,刘红2,3,张福仁2,3   

  1. 1山东大学齐鲁医学院公共卫生学院,山东济南,250012;2山东第一医科大学附属皮肤病医院,山东济南,250022;3山东省皮肤病性病防治研究所,山东济南,250022
  • 出版日期:2026-04-15 发布日期:2026-04-07

A bayesian model-based estimation of the global incidence and disease burden of pemphigus

LI Yang1,2,3, WANG Zhenzhen2,3, XIE Lanning1,2,3, LI Wenchao2,3, LIU Hong2,3, ZHANG Furen2,3   

  1. 1 School of Public Health, Cheeloo College of Medicine, Shandong University, Jinan 250012, China; 2 Dermatology Hospital of Shandong First Medical University, Jinan 250022, China; 3 Shandong Provincial Institute of Dermatology and Venereology, Shandong Academy of Medical Sciences, Jinan 250022, China
  • Online:2026-04-15 Published:2026-04-07

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摘要： 目的：天疱疮是一组罕见的自身免疫性大疱性疾病，其流行病学数据在全球范围内仍较匮乏。本研究旨在定量估计天疱疮及其亚型寻常型天疱疮（PV）在全球、区域和特定国家的流行病学负担。方法：系统检索天疱疮的流行病学研究，构建贝叶斯层次线性混合模型，估计天疱疮及PV在全球、区域和特定国家的发病率与新发病例数，并对患病率做描述性总结。结果：共纳入55项研究,模型预测，天疱疮与PV的全球发病率分别为4.05（0.84~8.25）/百万人年和3.62（0.73~7.60）/百万人年，年新发病例数分别约为3.25万与2.90万例。天疱疮的区域发病率从撒哈拉以南非洲南部1.92/百万人年至北非及中东5.32/百万人年不等；国家层面以伊朗最高（9.78/百万人年），摩洛哥最低（0.70/百万人年），PV亦呈现显著地理差异。全球女性天疱疮发病率是男性的1.36倍（4.18/百万人年 vs. 3.07/百万人年），PV为1.38倍（3.57/百万人年 vs. 2.59/百万人年）。成人发病率与全人群无显著差异，提示年龄分层数据不足。患病率亦呈地域异质性。结论：全球近84%的国家缺乏天疱疮流行病学数据，疾病分布存在明显的性别、年龄与地理差异。本研究填补了数据空白，为疾病监测与防控提供了依据。

关键词: 天疱疮, 寻常型天疱疮, 流行病学, 发病率, 贝叶斯层次线性混合模型

Abstract: Objective: Pemphigus is a rare group of autoimmune bullous diseases yet its epidemiological data remain limited worldwide. This study aimed to quantitatively estimate the global, regional, and national epidemiological burden of pemphigus and its subtype pemphigus vulgaris (PV). Methods: We systematically retrieved epidemiological studies on pemphigus and constructed a Bayesian hierarchical linear mixed model to estimate the incidence and annual new cases of pemphigus and PV globally, regionally, and nationally. The prevalence was summarized descriptively. Results: Fifty-five studies were included. The estimated global incidence of pemphigus and PV was 4.05 (0.84-8.25) per million person-years and 3.62 (0.73-7.60) per million person-years, with approximately 32 500 and 29 000 new cases per year, respectively. The regional incidence of pemphigus varied from 1.92 per million person-years in southern Sub-Saharan Africa to 5.32 in North Africa and the Middle East. At the national level, Iran had the highest incidence (9.78 per million person-years), while Morocco had the lowest (0.70 per million person-years). A similar geographic pattern was observed for PV. Females had a consistently higher incidence globally, with a 1.36-fold difference for pemphigus (4.18 per million person-years vs. 3.07 per million person-years) and a 1.38-fold difference for PV (3.57 per million person-years vs. 2.59 per million person-years). No significant difference was found between adult and general population incidence, suggesting insufficient age-stratified data. Prevalence also showed considerable geographical heterogeneity. Conclusion: Epidemiological data on pemphigus are lacking in nearly 84% of countries worldwide, with notable disparities by sex, age, and geography. This study fills critical data gaps and provides evidence for disease surveillance and prevention.

Key words: pemphigus, pemphigus vulgaris, epidemiology, incidence, bayesian hierarchical linear mixed model