Pulmonary Fibrosis in Generalized Scleroderma

Report of a Case and Review of the Literature

¹ The Department of Medicine and Surgery, Veterans Administration Hospital, Oteen, North Carolina.

A case of generalized progressive scleroderma, with roentgenologically visualized pulmonary involvement, is reported, and the essential features of such cases that have been reported in the English literature are tabulated and discussed.

Scleroderma of the lungs may clinically antedate the development of typical skin changes and present the picture of chronic pulmonary disease. This occurred in approximately one-fourth of the cases reviewed.

Roentgenograms present a picture of net-like interstitial fibrosis of the lower two-thirds of the lungs, sometimes associated with multiple varying sized cysts.

The fundamental pathological changes consist of diffuse alveolar wall sclerosis and alveolar obliteration, cystic changes, in some cases, and vascular narrowing due to involvement of the supporting connective tissue of the vessel walls.

In obscure cases of chronic interstitial fibrosis of the lung, generalized progressive scleroderma should be kept in mind.

The para-aminobenzoic acid used in the treatment of our case was furnished by Wyeth, Incorporated, Philadelphia, Pennsylvania.