Pulmonary function tests, high-resolution computerized tomography, [alpha]^sub 1^-antitrypsin measurement, and early detection of pulmonary involvement in patients with systemic sclerosis

Objective. Pulmonary disease represents a major complication of systemic sclerosis (SSc). However, pulmonary involvement is commonly silent. In this study, we investigated the relationship between serum α^sub 1^-antitrypsin and other means of assessing pulmonary involvement. Methods. Twenty-two patients affected by SSc were studied (mean age 37.6±14.3 years, mean duration of disease 9.9±11.9 years). Fourteen had the diffuse form of disease (dSSc) and eight had the limited form (lSSc). All patients underwent pulmonary function tests, high-resolution computed tomography (HRCT) of the lungs, echocardiography, and serum assessment of α^sub 1^-antitrypsin. Results. Mean percentage of predicted values of forced vital capacity was lower in patients with dSSc than with lSSc (72.3±17.8 vs 74.5±8, P=NS). Mean percentage of predicted values of forced expiratory volume in 1-s forced vital capacity (FEV1/FVC) was lower in patients with lSSc (79.8±7.5 for lSSc vs 84.4±7.8 for dSSc, P=NS). The overall HRCT score was 5.6±5.9 with no significant difference between disease subgroups. Pulmonary hypertension was detected in two cases, both with dSSc. Alpha^sub 1^-antitrypsin was significantly higher in patients than in controls (P