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Current Respiratory Medicine Reviews

Editor-in-Chief

ISSN (Print): 1573-398X
ISSN (Online): 1875-6387

Systemic Sclerosis Associated Interstitial Lung Disease

Author(s): Virginia Steen and Nargues Weir

Volume 11, Issue 2, 2015

Page: [110 - 118] Pages: 9

DOI: 10.2174/1573398X11666150619183832

Price: $65

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.

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