Pulmonary Manifestations of Scleroderma

SCLERODERMA was once thought to be a disease limited to the skin, but recent reports have emphasized involvement of the gastrointestinal tract,1heart,2lungs,3kidneys,4endocrine glands, bones, and skeletal muscle.1It is now thought that scleroderma is a systemic disease, and Goetz5has suggested that a more appropriate name would be progressive systemic sclerosis, of which skin involvement is only one manifestation. In the early descriptions of generalized scleroderma, dyspnea and a tendency to pulmonary infections were attributed to restrictions of respiratory movements produced by sclerotic changes in the skin and muscles of the chest wall. Now it seems more likely that the pulmonary symptoms are due to fibrotic changes in the lungs. In 1891 Finlay6recognized the association between scleroderma and pulmonary fibrosis, while Lewin and Heller,7in 1895, and later Matsui8demonstrated pulmonary fibrosis at autopsy in cases of scleroderma. The roentgenological featuresof pulmonary fibrosis