Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention

Aryeh Fischer,1 Nina M Patel,2 Elizabeth R Volkmann3 1Division of Rheumatology, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado School of Medicine, Denver, CO, USA; 2Division of Pulmonary, Allergy and Critical Care Medicine, Columbia Universi...

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Autores principales:	Fischer A, Patel NM, Volkmann ER
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2019
Materias:	systemic sclerosis interstitial lung diseases early diagnosis disease progression treatment outcome Diseases of the musculoskeletal system RC925-935
Acceso en línea:	https://doaj.org/article/2c548f2fa33040cb9dc89b82735b64d3
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spelling	oai:doaj.org-article:2c548f2fa33040cb9dc89b82735b64d32021-12-02T05:51:04ZInterstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention1179-156Xhttps://doaj.org/article/2c548f2fa33040cb9dc89b82735b64d32019-12-01T00:00:00Zhttps://www.dovepress.com/interstitial-lung-disease-in-systemic-sclerosis-focus-on-early-detecti-peer-reviewed-article-OARRRhttps://doaj.org/toc/1179-156XAryeh Fischer,1 Nina M Patel,2 Elizabeth R Volkmann3 1Division of Rheumatology, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado School of Medicine, Denver, CO, USA; 2Division of Pulmonary, Allergy and Critical Care Medicine, Columbia University Irving Medical Center, New York, NY, USA; 3Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USACorrespondence: Elizabeth R VolkmannDivision of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, 1000 Veteran Avenue, Suite 32-59, Los Angeles, CA 90095, USATel +1 310-825-2448Fax +1 310-206-6553Email EVolkmann@mednet.ucla.eduAbstract: Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.Keywords: systemic sclerosis, interstitial lung diseases, early diagnosis, disease progression, treatment outcomeFischer APatel NMVolkmann ERDove Medical Pressarticlesystemic sclerosisinterstitial lung diseasesearly diagnosisdisease progressiontreatment outcomeDiseases of the musculoskeletal systemRC925-935ENOpen Access Rheumatology: Research and Reviews, Vol Volume 11, Pp 283-307 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	systemic sclerosis interstitial lung diseases early diagnosis disease progression treatment outcome Diseases of the musculoskeletal system RC925-935
spellingShingle	systemic sclerosis interstitial lung diseases early diagnosis disease progression treatment outcome Diseases of the musculoskeletal system RC925-935 Fischer A Patel NM Volkmann ER Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
description	Aryeh Fischer,1 Nina M Patel,2 Elizabeth R Volkmann3 1Division of Rheumatology, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado School of Medicine, Denver, CO, USA; 2Division of Pulmonary, Allergy and Critical Care Medicine, Columbia University Irving Medical Center, New York, NY, USA; 3Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USACorrespondence: Elizabeth R VolkmannDivision of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, 1000 Veteran Avenue, Suite 32-59, Los Angeles, CA 90095, USATel +1 310-825-2448Fax +1 310-206-6553Email EVolkmann@mednet.ucla.eduAbstract: Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.Keywords: systemic sclerosis, interstitial lung diseases, early diagnosis, disease progression, treatment outcome
format	article
author	Fischer A Patel NM Volkmann ER
author_facet	Fischer A Patel NM Volkmann ER
author_sort	Fischer A
title	Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
title_short	Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
title_full	Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
title_fullStr	Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
title_full_unstemmed	Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention
title_sort	interstitial lung disease in systemic sclerosis: focus on early detection and intervention
publisher	Dove Medical Press
publishDate	2019
url	https://doaj.org/article/2c548f2fa33040cb9dc89b82735b64d3
work_keys_str_mv	AT fischera interstitiallungdiseaseinsystemicsclerosisfocusonearlydetectionandintervention AT patelnm interstitiallungdiseaseinsystemicsclerosisfocusonearlydetectionandintervention AT volkmanner interstitiallungdiseaseinsystemicsclerosisfocusonearlydetectionandintervention
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Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention

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