Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.
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.
Johnson
Sindhu R
SR
Division of Rheumatology, Department of Pathology, University Health Network, University of Toronto, Toronto, Ontario, Canada.
Patsios
Demetris
D
Hwang
David M
DM
Granton
John T
JT
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*