Pulmonary vascular disease in systemic sclerosis (scleroderma): Treatment
- John Varga, MD
John Varga, MD
- John and Nancy Hughes Professor of Medicine
- Northwestern University Feinberg School of Medicine
- Virginia Steen, MD
Virginia Steen, MD
- Professor of Medicine
- Georgetown University Medical Center
- Paul Hassoun, MD
Paul Hassoun, MD
- Professor of Medicine
- Department of Medicine, Johns Hopkins University
- Section Editors
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- John S Axford, DSc, MD, FRCP, FRCPCH
John S Axford, DSc, MD, FRCP, FRCPCH
- Section Editor — Scleroderma
- Emeritus Professor of Rheumatology
- St George's University of London
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 PAHTo continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- GENERAL MEASURES
- Supportive therapies
- PULMONARY ARTERIAL HYPERTENSION-DIRECTED THERAPY
- Calcium channel blockers
- Endothelin-1 receptor antagonists
- - Nonselective
- - Selective
- Nitric oxide-cyclic guanosine monophosphate enhancers
- - Phosphodiesterase type 5 inhibitors
- - Guanylate cyclase stimulant
- Prostacyclin pathway agonists
- - Epoprostenol
- - Treprostinil
- - Iloprost
- - Selexipag
- Combination therapy
- LUNG TRANSPLANTATION
- SUMMARY AND RECOMMENDATIONS