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Updated: Feb 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Systemic sclerosis
1Service de médecine interne, hôpital Cochin, Centre de référence national pour les maladies systémiques auto-immunes rares, DHU Authors, Assistance publique-Hôpitaux de Paris, Paris, France.
Abstract:
Systemic sclerosis. Systemic sclerosis is more frequent among women (sex ratio from 3/1 to 8/1) and its prevalence varies from 30 to 240 per millions. Two types of SSc are described depending on the extent of skin involvement : limited and diffuse SSc. Clinical manifestations include fibrosing lesions (skin and pulmonary fibrosis) and vascular abnormalities (Raynaud's phenomenon and pulmonary arterial hypertension (PAH)). Auto-antibodies have been identified in SSc patients, directed at centromere proteins, topoisomerase 1 or RNA polymerase III. SSc is associated with a poor outcome particularly in diffuse forms associated with pulmonary involvement (pulmonary fibrosis and/or PAH). SSc treatment associate symptomatic treatments as well as treatment of visceral complications (PAH, renal crisis) and immunosuppressive therapy in patients with recent onset diffuse SSc and/or rapidely progressing interstitial lung disease.
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