Good Samuel D, Lee Ju Young, Johnson Robert E, Volkmann Elizabeth R
Division of Rheumatology, Department of Medicine, David Geffen School of Medicine.
David Geffen School of Medicine.
Curr Opin Rheumatol. 2025 Mar 1;37(2):103-112. doi: 10.1097/BOR.0000000000001063. Epub 2024 Oct 23.
Updates from large, observational cohorts and new statistical techniques have resulted in new data on the epidemiology of systemic sclerosis (SSc). This scoping review uses data from 2018 to 2024 to describe the current understanding of the epidemiology of SSc and several of its organ- manifestations.
Our review identified new estimates for the global incidence and prevalence of SSc (1.4-8.6 per 100 000 person-years and 17.6-18.9 per 100 000 individuals, respectively). Mortality rates remain high, though mortality at younger ages has decreased. interstitial lung disease and pulmonary arterial hypertension remain the most common causes of death for patients with SSc. Literature on gastrointestinal (GI) manifestations of SSc was scarce, and we identified significant heterogeneity in results. Furthermore, data on the epidemiology of racial, ethnic and sex-based disparities was lacking.
New techniques for the evaluation of the epidemiology of SSc highlight the high morbidity and mortality of SSc, and a growing prevalence rate compared with prior eras. Further research is needed to address notable heterogeneity in the reporting of epidemiological data and understudied disease manifestations, including GI disease and health disparities in disease outcomes.
大型观察性队列研究的更新以及新的统计技术带来了系统性硬化症（SSc）流行病学的新数据。本范围综述利用2018年至2024年的数据来描述目前对SSc流行病学及其一些器官表现的理解。
我们的综述确定了SSc全球发病率和患病率的新估计值（分别为每10万人年1.4 - 8.6例和每10万人17.6 - 18.9例）。死亡率仍然很高，不过年轻患者的死亡率有所下降。间质性肺疾病和肺动脉高压仍然是SSc患者最常见的死亡原因。关于SSc胃肠道（GI）表现的文献很少，并且我们发现结果存在显著异质性。此外，缺乏关于种族、民族和性别差异流行病学的数据。
评估SSc流行病学的新技术凸显了SSc的高发病率和死亡率，以及与之前时代相比不断上升的患病率。需要进一步研究以解决流行病学数据报告中显著的异质性以及研究不足的疾病表现，包括胃肠道疾病和疾病结局方面的健康差异。
