Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis
- 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 common, and are the leading cause of SSc-related death. The most common pulmonary manifestations of SSc are pulmonary hypertension (PH), interstitial lung disease (ILD), and any combination thereof.
The classification, definition, risk factors, screening, and prognosis of SSc-associated pulmonary arterial hypertension (PAH), specifically group 1 PAH, are reviewed here. Group 2 PH and group 3 PH are discussed separately. (See "Pulmonary hypertension associated with interstitial lung disease" and "Treatment of pulmonary hypertension in adults", section on 'Group 2 PH' and "Treatment of pulmonary hypertension in adults", section on 'Group 3 PH'.)
The evaluation and diagnosis of lung disease in SSc as well as clinical manifestations, diagnosis, and treatment of SSc-associated pulmonary arterial hypertension are discussed elsewhere. (See "Overview of pulmonary complications of systemic sclerosis (scleroderma)" and "Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in systemic sclerosis (scleroderma)" and "Pulmonary vascular disease in systemic sclerosis (scleroderma): Treatment" and "Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma)".)
Many different forms of pulmonary hypertension (PH) occur in systemic sclerosis (SSc). The World Health Organization (WHO) classifies patients with PH into five groups, as shown in the table (table 1) . SSc is most often associated with group 1 pulmonary arterial hypertension (PAH) and group 3 PH, and less commonly with group 2 PH. Due to the varied and sometimes mixed etiology underlying PH in SSc, the precise classification of the type of PH can be challenging.
The following is a brief description of the WHO classification of PH:
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- WHO CLASSIFICATION
- RISK FACTORS
- - Long-standing SSc
- - Limited cutaneous SSc
- Abnormal pulmonary function (low diffusion)
- Our approach
- Right heart catheterization
- SUMMARY AND RECOMMENDATIONS