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Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in 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


Interstitial lung disease (ILD) is one of the major types of lung involvement in systemic sclerosis (SSc). SSc-associated ILD consists of various histopathologic subtypes, most commonly nonspecific interstitial pneumonitis and usual interstitial pneumonitis. Affected patients have a worse prognosis than patients with SSc who are free of pulmonary involvement.

The clinical manifestations, evaluation, and diagnosis of SSc-associated ILD are discussed here. The treatment of SSc-associated ILD, the evaluation and management of pulmonary arterial hypertension and other pulmonary complications of SSc are discussed separately. (See "Overview of pulmonary complications of 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 subsets of SSc are commonly recognized and are distinguished by the extent of skin sclerosis and pattern of organ involvement. The type of cutaneous involvement has some correlation with the risk for ILD, as described below. (See "Overview and classification of scleroderma disorders", section on 'Limited and diffuse cutaneous SSc' and "Overview of pulmonary complications of systemic sclerosis (scleroderma)", section on 'Influence of diffuse cutaneous and limited cutaneous disease'.)

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). Patients with diffuse cutaneous SSc are at an increased risk of developing ILD early in the course of their disease [1].

Limited cutaneous SSc is characterized by skin sclerosis restricted to the hands, distal extremities, and, to a lesser extent, the face and neck. Patients with limited cutaneous SSc are less likely to develop ILD early in the course of disease, but may develop ILD as a late manifestation [1,2].

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