Ruaro Barbara, Confalonieri Marco, Matucci-Cerinic Marco, Salton Francesco, Confalonieri Paola, Santagiuliana Mario, Citton Gloria Maria, Baratella Elisa, Bruni Cosimo
Department of Pulmonology, University Hospital of Cattinara, 34149 Trieste TS, Italy.
Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, 50121 Firenze FI, Italy.
Pharmaceuticals (Basel). 2021 Feb 13;14(2):154. doi: 10.3390/ph14020154.
Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%. African Americans and subjects with diffuse cutaneous SSc or anti-topoisomerase 1 antibodies are most commonly affected. Currently, early ILD diagnosis can be made, and it is pivotal to improve the prognosis. The diagnostic mainstay test for SSc-ILD is high-resolution computed tomography for the morphology and pulmonary function tests for the functional aspects. Treatment planning and intensity are guided by the disease severity and risk of progression. Traditionally, therapy has depended on combinations of immunosuppressants, particularly cyclophosphamide and mycophenolate mofetil, which can be supplemented by targeted biological and antifibrotic therapies. Benefits have been observed in trials on hematopoietic autologous stem cell transplantation for patients with progressive SSc, whilst lung transplantation is reserved for refractory SSc-ILD cases. Herein, recent advances in SSc-ILD treatment will be explored.
系统性硬化症（SSc）患者常受间质性肺疾病（ILD）影响，尽管近期治疗取得了进展，但ILD仍是SSc患者的主要死因，10年死亡率高达40%。非裔美国人以及弥漫性皮肤型SSc或抗拓扑异构酶1抗体阳性的患者受影响最为常见。目前，ILD能够实现早期诊断，改善预后至关重要。SSc-ILD的主要诊断性检查是用于评估形态学的高分辨率计算机断层扫描以及用于评估功能方面的肺功能测试。治疗方案的制定和治疗强度取决于疾病的严重程度和进展风险。传统上，治疗依赖于免疫抑制剂的联合使用，尤其是环磷酰胺和霉酚酸酯，还可辅以靶向生物治疗和抗纤维化治疗。对于进行性SSc患者，造血自体干细胞移植试验已观察到疗效，而肺移植则适用于难治性SSc-ILD病例。本文将探讨SSc-ILD治疗的最新进展。
