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Updated: Jul 9, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Interstitial lung disease as the first manifestation of systemic sclerosis
R van der Kamp1, P P Tak, H M Jansen
1Department of Pulmonology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands.
Abstract:
We describe three patients with progressive fibrosing interstitial lung disease (ILD) as the first and only manifestation of systemic sclerosis. In one patient the presence of anti-Scl-70 autoantibodies suggested systemic sclerosis to be the underlying cause of the disease. In the two other subjects, however, anti-Scl-70 antibodies were negative. In these patients the lung disease preceded other manifestations of systemic sclerosis by several years. Diagnosis, prognosis and treatment of systemic sclerosisassociated ILD is discussed.
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