Among 5 patients with idiopathic diffuse interstitial disease and 16 patients with scleroderma, one with idiopathic pulmonary fibrosis died within six months of follow-up examination. Three others showed progressive deterioration of lung volumes and diffusing capacity and also died. One patient who had a relatively acute interstitial pneumonitis had remarkable clinical and functional remission with corticosteroid therapy.
Of the 16 patients with scleroderma, 13 had evidence of interstitial fibrosis and 2 of peribronchial fibrosis. However, progressive reduction of lung volumes and diffusing capacity with time was seen in only three. Improvement with therapy was seen in two, while one patient's condition was thought to represent clearing of pleurisy, and the other's improvement of bronchial obstruction.
Interstitial fibrosis in scleroderma appears to have a more indolent course than the idiopathic variety, but shows little or no response to treatment.