Raynaud's Disease and Primary Pulmonary Hypertension

Abstract

A case of Raynaud's disease with autopsy is reported in which the clinical picture was dominated by pulmonary hypertension apparently unassociated with any significant pulmonary parenchymal disease. Microscopically cellular intimal proliferation of small pulmonary muscular arteries and arterioles was the most characteristic lesion. In several proximal medium-sized muscular arteries there was necrotizing arteritis with thrombus formation. Similar vascular lesions were not found in other organs, although generalized arteriosclerosis and arteriolosclerosis were present. The pulmonary vascular changes are thought to represent a local exacerbation of generalized vascular disease, but a specific etiology was not apparent.