[Pulmonary functions in patients with progressive systemic sclerosis]

We studied the correlations of pulmonary function with clinical symptoms or autoantibody profiles in patients with progressive systemic sclerosis (PSS). Percent vital capacity (% VC) was low in 40% of PSS patients and percent diffusing capacity of carbon oxide (% DLCO) was decreased in 76% of the patients. Eighty-five percent of the patients had abnormal pulmonary functions. The % DLCO was significantly low in male patients compared with female patients. The arterial partial pressure of oxygen (PO2) in patients with esophageal hypomotility was significantly lower than those without esophageal hypomotility. PSS with Sjögren's syndrome (SS) showed a significant decrease of the % VC (p < 0.05) and PO2 (p < 0.05), and of lung fibrosis was frequent in the patients compared with those without SS. Pulmonary fibrosis was recognized in 63% of PSS patients and was more frequent in the patients with anti-Scl-70 antibody than those without the antibody. The annual decrease of VC was 62 ml in PSS patients, and that of DLco was 0.12 ml/min/mmHg. The annual decrease of VC was more prominent in the PSS patients with severe pulmonary fibrosis or anti-Scl-70 antibody. The decrease of VC, however, was mild in the patients with anti-RNP antibody compared with those without the antibody.