Systemic sclerosis: A comprehensive systematic review of global epidemiology, sex and ethnic disparities, disease burden, and organ-specific involvement

IF 7 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-05-25 DOI:10.1016/j.berh.2026.102156

Carlos Enrique Toro-Gutierrez , Gerardo Quintana-Lopez , Janet Pope

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Abstract

The global epidemiology, regional variation, sex- and ethnicity-based disparities, disease burden, and organ-specific involvement in systemic sclerosis were reviewed. A qualitative systematic search of PubMed/MEDLINE, EMBASE, SciELO, and the Cochrane Library was conducted following PRISMA 2020 guidelines.

Forty-seven studies reporting incidence, prevalence, organ involvement, mortality, or quality of life were included. Global prevalence ranged from 0.9 to 37.9 per 100,000 and incidence from 0.05 to 4.1 per 100,000 person-years, with marked geographic heterogeneity across the globe.

Women were predominantly affected (4-6:1), whereas men showed more severe disease and higher mortality.

African-descent, Indigenous American, and some South Asian populations had higher frequencies of diffuse cutaneous systemic sclerosis, interstitial lung disease, and reduced survival. Pulmonary involvement accounted for most disease burden, with interstitial lung disease in 35-55% and pulmonary arterial hypertension in 6-12%.

These findings highlight substantial epidemiologic heterogeneity and the need for early detection and standardized care in expert centers.

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系统性硬化症：全球流行病学、性别和种族差异、疾病负担和器官特异性受累的全面系统综述。

综述了系统性硬化症的全球流行病学、地区差异、基于性别和种族的差异、疾病负担和器官特异性受累。按照PRISMA 2020指南对PubMed/MEDLINE、EMBASE、SciELO和Cochrane图书馆进行定性系统检索。纳入了47项报告发病率、患病率、器官受累、死亡率或生活质量的研究。全球流行率为每10万人0.9至37.9人，发病率为每10万人年0.05至4.1人，全球各地存在明显的地理异质性。受影响的主要是妇女（4-6:1），而男子则表现出更严重的疾病和更高的死亡率。非洲裔、美洲原住民和一些南亚人群弥漫性皮肤系统性硬化症、间质性肺病的发病率较高，生存率较低。肺受累占疾病负担的大部分，间质性肺病占35-55%，肺动脉高压占6-12%。这些发现强调了大量的流行病学异质性以及在专家中心进行早期发现和标准化护理的必要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Best Practice & Research in Clinical Rheumatology 医学-风湿病学

CiteScore

9.40

自引率

0.00%

发文量

审稿时长

27 days

期刊介绍： Evidence-based updates of best clinical practice across the spectrum of musculoskeletal conditions. Best Practice & Research: Clinical Rheumatology keeps the clinician or trainee informed of the latest developments and current recommended practice in the rapidly advancing fields of musculoskeletal conditions and science. The series provides a continuous update of current clinical practice. It is a topical serial publication that covers the spectrum of musculoskeletal conditions in a 4-year cycle. Each topic-based issue contains around 200 pages of practical, evidence-based review articles, which integrate the results from the latest original research with current clinical practice and thinking to provide a continuous update. Each issue follows a problem-orientated approach that focuses on the key questions to be addressed, clearly defining what is known and not known. The review articles seek to address the clinical issues of diagnosis, treatment and patient management. Management is described in practical terms so that it can be applied to the individual patient. The serial is aimed at the physician in both practice and training.