Abstract
Systemic sclerosis is a disease of typically middle aged women, with significant morbidity and mortality predominantly caused by lung involvement. Screening for and diagnosis of interstitial lung disease is critical given the decreased survival associated with progressive fibrotic lung disease. Monitoring of disease is necessary once the diagnosis has been made, and surgical lung biopsy is usually unnecessary. Treatment centers on immunosuppressants without additional steroids with attention to identification and management of co-existing complications, such as pulmonary hypertension and gastro-esophageal reflux, as well as supportive therapy with supplemental oxygen and exercise. Referral for lung transplant evaluation should be considered when there is persistent progression.
Keywords: Fibrosis, interstitial lung disease (ILD), review, scleroderma, severe.
Current Respiratory Medicine Reviews
Title:Systemic Sclerosis Associated Interstitial Lung Disease
Volume: 11 Issue: 2
Author(s): Virginia Steen and Nargues Weir
Affiliation:
Keywords: Fibrosis, interstitial lung disease (ILD), review, scleroderma, severe.
Abstract: Systemic sclerosis is a disease of typically middle aged women, with significant morbidity and mortality predominantly caused by lung involvement. Screening for and diagnosis of interstitial lung disease is critical given the decreased survival associated with progressive fibrotic lung disease. Monitoring of disease is necessary once the diagnosis has been made, and surgical lung biopsy is usually unnecessary. Treatment centers on immunosuppressants without additional steroids with attention to identification and management of co-existing complications, such as pulmonary hypertension and gastro-esophageal reflux, as well as supportive therapy with supplemental oxygen and exercise. Referral for lung transplant evaluation should be considered when there is persistent progression.
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Cite this article as:
Steen Virginia and Weir Nargues, Systemic Sclerosis Associated Interstitial Lung Disease, Current Respiratory Medicine Reviews 2015; 11 (2) . https://dx.doi.org/10.2174/1573398X11666150619183832
DOI https://dx.doi.org/10.2174/1573398X11666150619183832 |
Print ISSN 1573-398X |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6387 |
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