Interstitial lung disease (ILD) affects the majority of patients with systemic sclerosis (SSc) and is the leading cause of death in SSc. Traditionally, treatments for SSc-ILD have targeted broad suppression of the immune system with agents such as cyclophosphamide and mycophenolate. The recently published Scleroderma Lung Study (SLS) II demonstrated that treatment with either oral cyclophosphamide or mycophenolate led to similar improvement in lung function, dyspnea and radiographic extent of fibrosis. However, with the emergence of anti-fibrotic therapy for the treatment of idiopathic pulmonary fibrosis, the repurposing of biologic agents used to treat other connective tissue diseases, and the introduction of hematopoetic stem-cell transplantation for patients with early diffuse SSc, options for managing SSc-ILD have increased. For the first time ever, patients and physicians have choices for how to treat SSc-ILD. At the same time, the study and administration of these novel therapeutic agents have raised important clinical research questions that warrant further investigation. This review describes the current and experimental therapies available for SSc-ILD. This review also explores unanswered questions directly relevant to patient care and future research efforts in this area, including questions on indications for initiation of SSc-ILD therapy, the use of maintenance SSc-ILD therapy and the duration of SSc-ILD therapy. Conducting studies designed to answer these important questions is central to advancing SSc-ILD research and improving outcomes for patients with this devastating disease.
J scleroderma relat disord 2017; 2(2): 72 - 83
Article Type: REVIEW
AuthorsElizabeth R. Volkmann, Augustine Chung, Donald P. Tashkin
- • Accepted on 23/03/2017
- • Available online on 25/04/2017
- • Published in print on 31/05/2017
This article is available as full text PDF.
- Volkmann, Elizabeth R. [PubMed] [Google Scholar]
- Chung, Augustine [PubMed] [Google Scholar]
- Tashkin, Donald P. [PubMed] [Google Scholar] , * Corresponding Author (DTashkin@mednet.ucla.edu)
Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles - USA