Analysis of 15 cases with progressive systemic sclerosis proved by histology is given. Data obtained by examinations on respiratory functions including respiratory mechanisms, were compared to radiologic pictures and clinical symptoms. Spirometry changes have been found to be essentially identical to those in the Hamman- Rich syndrome, except for the elastic recoil pressure diagram. The loss of value of transpulmonal pressure at maximal inspiration (Pimax) compared with the same parameter in cases of pulmonary fibrosis with a similar decrease of vital capacity was particularly marked. A probable explanation of this phenomenon could be that atrophy of breathing muscles is one component of the disease. This would have the result that the muscular system is not able to overcome the elastic resistance of the rigid lung. The comparison of the values of vital capacity to those of Pi max would provide a possibility to separate pulmonary and extra-pulmonary components of respiratory function loss

Keywords:
Progressive systemic sclerosis, Pulmonary fibrosis, Respiratory mechanism examination, Elastic recoil pressure, Spirometric examination
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© 1973 S. Karger AG, Basel
1973
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