Humair Grégoire, Daccord Cécile, Beigelman-Aubry Catherine, Lazor Romain
Service de pneumologie, CHUV, 1011 Lausanne.
Service de radiodiagnostic et radiologie interventionnelle, CHUV, 1011 Lausanne.
Rev Med Suisse. 2020 Nov 18;16(715):2218-2223.
Interstitial lung disease is a frequent complication of systemic sclerosis and has now become the leading cause of death in this disorder. It mainly occurs during the first five years after the diagnosis of systemic sclerosis. Various risk factors are associated with the occurrence of interstitial lung disease, including the presence of anti-topoisomerase I antibodies (Scl-70) and the diffuse cutaneous form of systemic sclerosis. The most common radio-pathological presentation is nonspecific interstitial pneumonia, followed by usual interstitial pneumonia. The classical immunosuppressive treatment of systemic sclerosis-associated interstitial lung disease is evolving, as recent studies suggest a beneficial effect of biological agents such as rituximab and tocilizumab, and antifibrotic drugs such as nintedanib.
间质性肺疾病是系统性硬化症的常见并发症，现已成为该疾病的主要死因。它主要发生在系统性硬化症诊断后的头五年内。多种危险因素与间质性肺疾病的发生有关，包括抗拓扑异构酶I抗体（Scl-70）的存在以及系统性硬化症的弥漫性皮肤型。最常见的放射病理学表现是非特异性间质性肺炎，其次是寻常型间质性肺炎。系统性硬化症相关间质性肺疾病的经典免疫抑制治疗正在不断发展，因为最近的研究表明利妥昔单抗和托珠单抗等生物制剂以及尼达尼布等抗纤维化药物具有有益作用。
