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Overview of pulmonary complications of systemic sclerosis (scleroderma)

John Varga, MD
Section Editors
Talmadge E King, Jr, MD
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Helen Hollingsworth, MD


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 [1]. 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".)


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].

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Literature review current through: Nov 2017. | This topic last updated: Jul 12, 2016.
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