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Pulmonary vascular disease in systemic sclerosis (scleroderma): Treatment

John Varga, MD
Virginia Steen, MD
Paul Hassoun, MD
Section Editors
Talmadge E King, Jr, MD
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Geraldine Finlay, MD


Pulmonary complications of systemic sclerosis (SSc) are both frequent and the leading cause of SSc-related death [1,2]. The most common pulmonary manifestations of SSc are the following:

Pulmonary arterial hypertension (PAH)

Interstitial lung disease (ILD)

Pulmonary hypertension (PH) due to ILD

A combination of ILD and PAH


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Literature review current through: Feb 2017. | This topic last updated: Mar 02, 2017.
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