title: Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.
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.
authors.affiliation: Division of Rheumatology, Department of Pathology, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
authors.forename: Demetris
authors.initials: D
authors.lastname: Patsios
authors.forename: David M
authors.initials: DM
authors.lastname: Hwang
authors.forename: John T
authors.initials: JT
authors.lastname: Granton
mesh_terms: D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008875:Middle Aged; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications*; D045745:Scleroderma, Limited / Q000150:complications*