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.

Sildenafil: from angina to erectile dysfunction to pulmonary hypertension and beyond

“…Patients with pulmonary arterial hypertension often complain about intermittent, temperature-dependent peripheral vasospasms resulting in perfusion deficiencies in their fingers and toes (socalled Raynaud's phenomenon) even in the absence of a proven collagen vascular disease [129][130][131] . When treated with vasodilators, these symptoms can improve in parallel with, but also independent of, improvements in pulmonary haemodynamics.…”

Raynaud's phenomenon. An update.

“…35 Another example of vasospasm in more than one circulatory bed is the occurrence of pulmonary hypertension with Raynaud's phenomenon but no underlying systemic disease. 36 Although vasospasm has not been demonstrated in the pulmonary vasculature of these patients, pathological abnormalities to account for the pulmonary hypertension are absent. Furthermore, patients with scleroderma may have Raynaud's phenomenon, decreased renal cortical blood flow with cooling or a cold pressor stimulus, 37 myocardial infarctions with normal coronary arteries, and left ventricular dysfunction with cold exposure.…”