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The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease
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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379834/ https://www.ncbi.nlm.nih.gov/pubmed/32703199 http://dx.doi.org/10.1186/s12931-020-01459-0 |
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author | Hoffmann-Vold, Anna-Maria Allanore, Yannick Bendstrup, Elisabeth Bruni, Cosimo Distler, Oliver Maher, Toby M. Wijsenbeek, Marlies Kreuter, Michael |
author_facet | Hoffmann-Vold, Anna-Maria Allanore, Yannick Bendstrup, Elisabeth Bruni, Cosimo Distler, Oliver Maher, Toby M. Wijsenbeek, Marlies Kreuter, Michael |
author_sort | Hoffmann-Vold, Anna-Maria |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7379834 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-73798342020-08-04 The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease Hoffmann-Vold, Anna-Maria Allanore, Yannick Bendstrup, Elisabeth Bruni, Cosimo Distler, Oliver Maher, Toby M. Wijsenbeek, Marlies Kreuter, Michael Respir Res Review 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. BioMed Central 2020-07-23 2020 /pmc/articles/PMC7379834/ /pubmed/32703199 http://dx.doi.org/10.1186/s12931-020-01459-0 Text en © The Author(s) 2020 Open AccessThis 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 give appropriate 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 Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission 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 the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Review Hoffmann-Vold, Anna-Maria Allanore, Yannick Bendstrup, Elisabeth Bruni, Cosimo Distler, Oliver Maher, Toby M. Wijsenbeek, Marlies Kreuter, Michael The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title | The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title_full | The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title_fullStr | The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title_full_unstemmed | The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title_short | The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease |
title_sort | need for a holistic approach for ssc-ild – achievements and ambiguity in a devastating disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379834/ https://www.ncbi.nlm.nih.gov/pubmed/32703199 http://dx.doi.org/10.1186/s12931-020-01459-0 |
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