Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease
File(s)
Published version
Author(s)
Distler, Oliver
Volkmann, Elizabeth R
Hoffmann-Vold, Anna Maria
Maher, Toby M
Type
Journal Article
Abstract
Introduction: Systemic sclerosis (SSc) is a rare and complex connective tissue disease characterized by fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is a common complication of SSc and the leading cause of SSc-related death. No drugs are licensed for the treatment of SSc-ILD. Areas covered: This review provides an overview of the current treatment of SSc-ILD and a perspective on investigational therapies, focusing on those studied in randomized controlled trials. Expert opinion: There is substantial room for improvement in the treatment of SSc-ILD. Current treatment focuses on immunosuppressant therapies, particularly cyclophosphamide and mycophenolate. Hematopoietic stem cell transplantation has been shown to improve long-term outcomes, but the risk of treatment-related mortality restricts its use to select patients at specialized centers. Modifying the course of disease to improve outcomes remains the goal for new therapies. Several drugs are under investigation as potential therapies for SSc-ILD, providing hope that the limited treatment armamentarium for SSc-ILD will be expanded and improved in the near future. Expert consensus is needed on how to screen for and monitor SSc-ILD and on when to initiate and escalate therapy.
Date Issued
2019-10-03
Date Acceptance
2019-09-12
Citation
Expert Review of Clinical Immunology, 2019, 15 (10), pp.1009-1017
ISSN
1744-666X
Publisher
Taylor & Francis
Start Page
1009
End Page
1017
Journal / Book Title
Expert Review of Clinical Immunology
Volume
15
Issue
10
Copyright Statement
© 2019 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way.
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/31566449
Subjects
Autoimmune diseases
connective tissue diseases
pulmonary fibrosis
rheumatic diseases
treatment
Publication Status
Published
Coverage Spatial
England
Date Publish Online
2019-09-30