Pulmonary involvement in progressive systemic sclerosis: a multidisciplinary approach.

In an attempt to define the early features and the natural evolution of lung involvement in progressive systemic sclerosis (PSS), we planned a multidisciplinary study of these patients using radiological, scintigraphic and functional methods. As yet we have studied 21 subjects, all affected by PSS according to the American Rheumatism Association criteria. A well-defined restrictive functional pattern was present only in 10 patients out of 21; an increased elastic recoil may be present before a significant decrease of the transfer test for carbon monoxide; an increased alveolar-capillary permeability assessed by the 99mTc-diethylene-triamine-pentacetic acid (99mTc-DTPA) clearance rate has been detected in almost all the patients (13 out of 14) in at least one lung field; no significant correlation has been found between the radiological lung involvement and the 99mTc-DTPA clearance rate. We think that these preliminary results are consistent with an alveolar and interstitial inflammation rather than with a vasculitic process.