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
- 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 PAH
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- PRIMARY THERAPY
- Treating systemic sclerosis
- Supportive therapies
- ADVANCED THERAPY
- 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