Overview of pulmonary complications of systemic sclerosis (scleroderma)
- John Varga, MD
John Varga, MD
- John and Nancy Hughes Professor of Medicine
- Northwestern University Feinberg School of Medicine
- 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 involvement occurs in over 80 percent of patients with systemic sclerosis (SSc) and is second in frequency only to esophageal involvement as a visceral complication . It has surpassed renal involvement as the most common cause of death.
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the most frequent major types of lung involvement. Affected patients have a poorer prognosis than those with SSc who are free of pulmonary involvement.
The spectrum of pulmonary complications of SSc and an approach to their evaluation will be reviewed here. The clinical manifestations, diagnosis, therapy, and prognosis of SSc-associated ILD and PAH are discussed separately. (See "Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in systemic sclerosis (scleroderma)" and "Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma)" and "Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis".)
INFLUENCE OF DIFFUSE CUTANEOUS AND LIMITED CUTANEOUS DISEASE
Two major types of SSc are commonly recognized and are based on whether the extent of skin involvement is limited or diffuse. The type of cutaneous involvement correlates with the risk for different types of lung involvement, although substantial overlap exists. (See "Overview and classification of scleroderma disorders" and "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults".)
●Diffuse cutaneous SSc is characterized by extensive skin involvement with extension of skin sclerosis proximal to the wrists (particularly over the proximal limbs and trunk but commonly sparing the upper back). Approximately one third of patients with SSc have diffuse skin involvement. Patients with diffuse skin involvement due to SSc have a greater likelihood of developing interstitial lung disease (ILD; and also cardiac and renal disease), compared with those who have limited cutaneous SSc [2-4].To 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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- INFLUENCE OF DIFFUSE CUTANEOUS AND LIMITED CUTANEOUS DISEASE
- TYPES OF PULMONARY INVOLVEMENT
- Interstitial lung disease
- Pulmonary vascular disease
- - Pulmonary hypertension
- - Thromboembolic disease
- - Pulmonary capillary hemangiomatosis and veno-occlusive disease
- Pleural effusion
- Spontaneous pneumothorax
- Recurrent aspiration
- Drug-associated pneumonitis
- Airways disease
- Lung cancer
- Respiratory muscle weakness
- EVALUATION FOR LUNG DISEASE AT TIME OF SSC PRESENTATION
- Clinical assessment
- Laboratory testing
- Pulmonary function testing
- SUBSEQUENT MONITORING
- DIAGNOSIS OF LUNG DISEASE IN SYSTEMIC SCLEROSIS
- Interstitial lung disease
- Pulmonary hypertension
- Other pulmonary complications
- SUMMARY AND RECOMMENDATIONS