Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.
case_series · Level IV
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- Record sourced from PubMed, PMID 16981286.
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Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect.
Medical subject headings
- Hypertension, Pulmonary
- Pulmonary Veno-Occlusive Disease
- Scleroderma, Limited