Pulmonary vascular changes in scleroderma

Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

“…Severe pulmonary hypertension is a well-recognized complication of a number of collagen vascular disorders, particularly the CREST syndrome (Salerni et ul., 1977;Smith et al, 1979;Trell and Lindstrom, 1971;Young and Mark, 1978). Although myocardial involvement, hypertension, and pulmonary fibrosis can also occur in scleroderma generally, and CREST in particular, pulmonary hypertension can occur independently (Botstein and LeRoy, 1981;Bulkley et al, 1976;Gaffrey et al, 1982;Salerni et al, 1977;Smith er al., 1979;Trell and Lindstrom, 1971;Young and Mark, 1978).…”

“…Although myocardial involvement, hypertension, and pulmonary fibrosis can also occur in scleroderma generally, and CREST in particular, pulmonary hypertension can occur independently (Botstein and LeRoy, 1981;Bulkley et al, 1976;Gaffrey et al, 1982;Salerni et al, 1977;Smith er al., 1979;Trell and Lindstrom, 1971;Young and Mark, 1978). Pulmonary hypertension frequently leads to progressive right heart failure and death (Salemi et a]., 1977).…”

Vascular reactivity and pulmonary hypertension in systemic sclerosis

“…Emphasis on the pathogenesis of systemic sclerosis has changed from that of an initial fibrotic process to primary small vessel disease (1,6). Pulmonary hypertension is a common finding in systemic sclerosis (7).…”

“…Pulmonary hypertension is a common finding in systemic sclerosis (7). In the absence of small vessel disease, there is little evidence that anything short of marked lung fibrosis leads to significant rises in pulmonary pressure (6,7). However, perivascular distribution of fibrous tissue has not been systematically quantified, and to attribute pulmonary hypertension solely to vascular disease requires near absence of fibrosis and the presence of appropriate histologic changes of the resistance vessels.…”

Scleroderma and pulmonary hypertension

“…In patients with limited scleroderma, formerly referred to as CREST (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasias), up to 60% of the patients have pulmonary hypertension (11,12,14,15,17) . While not all patients have clinically significant pulmonary hypertension, two thirds of the patients with scleroderma will have pathologic evidence of pulmonary vascular disease (17,18) . Stupai reported 2-year survival in patients with CREST without pulmonary hypertension to be greater than 80%, while patients with pulmonary hypertension had a 2-year survival of 40% (11) .…”