Pulmonary hypertension was detected in two cases, both with dSSc. No significant correlation was observed between skin score or degree of dyspnea with HRCT score, lung volume, or carbon monoxide diffusing capacity. Restrictive lung disease was more pronounced in patients with dSSc. Alpha 1 -antitrypsin levels correlated significantly with ground glass opacities, an early finding of pulmonary involvement in SSc. Extent and severity of skin involvement and degree of dyspnea were not related to pulmonary involvement.
Few data exist on the relationship between pulmonary and extrapulmonary involvement in SSc.
Chest radiography has a limited role in the detection of earlier stages of lung involvement, and pulmonary function studies are of paramount importance in early detection. Alpha 1 -antitrypsin is a glycoprotein. The aim of this study was to assess pulmonary involvement in patients with systemic sclerosis and the correlation between conventional tests and serum 1 -antitrypsin levels. All patients had complete clinical examination including evaluation for gastrointestinal, pulmonary, cardiac, renal, and muscle involvement. Esophageal manometric studies were carried out on all patients.