The need for a holistic approach for SSc-ILD - achievements and ambiguity in a devastating disease
File(s)Hoffmann-Voldet al. Respiratory Research.pdf (1.06 MB)
Published version
Author(s)
Type
Journal Article
Abstract
Systemic sclerosis (SSc) is a multi-organ autoimmune disease with complex interactions between immune-mediated inflammatory processes and vascular pathology leading to small vessel obliteration, promoting uncontrolled fibrosis of skin and internal organs. Interstitial lung disease (ILD) is a common but highly variable manifestation of SSc and is associated with high morbidity and mortality. Treatment approaches have focused on immunosuppressive therapies, which have shown some efficacy on lung function. Recently, a large phase 3 trial showed that treatment with nintedanib was associated with a reduction in lung function decline. None of the conducted randomized clinical trials have so far shown convincing efficacy on other outcome measures including quality of life determined by patient reported outcomes. Little evidence is available for non-pharmacological treatment and supportive care specifically for SSc-ILD patients, including pulmonary rehabilitation, supplemental oxygen, symptom relief and adequate information. Improved management of SSc-ILD patients based on a holistic approach is necessary to support patients in maintaining as much quality of life as possible throughout the disease course and to improve long-term outcomes.
Date Issued
2020-07-23
Date Acceptance
2020-07-17
Citation
Respiratory Research, 2020, 21 (1)
ISSN
1465-9921
Publisher
BioMed Central
Journal / Book Title
Respiratory Research
Volume
21
Issue
1
Copyright Statement
© The Author(s). 2020. This article is licensed under a Creative Commons Attribution 4.0 International License,which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you giveappropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commonslicence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commonslicence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtainpermission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to thedata made available in this article, unless otherwise stated in a credit line to the data.
License URL
Sponsor
British Lung Foundation
National Institute for Health Research
British Lung Foundation
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/32703199
PII: 10.1186/s12931-020-01459-0
Grant Number
BLF-RMF 15-16
CS-2013-13-017
C17-3
Subjects
Fibrosis
Interstitial lung disease
Systemic sclerosis
Treatment
Publication Status
Published
Coverage Spatial
England
Article Number
ARTN 197