Abstract

Nine cases of systemic scleroderma are described in which were found extensive pulmonary arterial lesions. It is postulated that these vascular lesions contributed to See Images in the PDF File the development of cor pulmonale in seven of the cases. The early vascular lesions had a loose, myxomatous character while late lesions were more compact and cellular. PAS and Alcian blue positive material in the early lesions suggested that carbohydrate-containing protein and mucopolysac-charide were present. The pulmonary vascular lesions have many pathogenic features in common with systemic vascular lesions and visceral lesions in the disorder.

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