Department of Rheumatology, University Hospital Zurich, University of Zurich, Schmelzbergstrasse 24, Zurich 8006, Switzerland; Division of Rheumatology, Department of Experimental Medicine, Careggi University Hospital - University of Florence, Florence, Italy.
Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Via Olgettina 60, Milan, Italy. Electronic address: https://twitter.com/CampochiaroCor.
Rheum Dis Clin North Am. 2023 May;49(2):279-293. doi: 10.1016/j.rdc.2023.01.005.
Systemic sclerosis-interstitial lung disease (SSc-ILD) is a major complication of SSc resulting in important morbidity and mortality. Next to cyclophosphamide and mycophenolate mofetil, tocilizumab and nintedanib have proven efficacy in the treatment of SSc-ILD. The highly variable course of SSc-ILD, the complexity in determining and predicting the progression of SSc-ILD, and the diversity of treatment options for SSc-ILD, pose many challenges for everyday clinical practice. In this review, currently available evidence for monitoring and treatment of SSc-ILD is summarized and areas where additional evidence is highly desirable are discussed.
系统性硬皮病-间质性肺病（SSc-ILD）是系统性硬皮病的主要并发症，导致重要的发病率和死亡率。除环磷酰胺和霉酚酸酯外，托珠单抗和尼达尼布已被证明可有效治疗 SSc-ILD。SSc-ILD 的病程变化很大，确定和预测 SSc-ILD 进展的复杂性，以及 SSc-ILD 的治疗选择多样性，给日常临床实践带来了许多挑战。在这篇综述中，总结了目前监测和治疗 SSc-ILD 的可用证据，并讨论了非常需要额外证据的领域。
