Issue: 2007 > November > case report

Interstitial lung disease as the first manifestation of systemic sclerosis

R. van der Kamp, P.P. Tak, H.M. Jansen, P. Bresser


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.