TY - JOUR T1 - Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension. JF - The Journal of Rheumatology JO - J Rheumatol SP - 2347 LP - 2350 VL - 33 IS - 11 AU - Sindhu R Johnson AU - Demetris Patsios AU - David M Hwang AU - John T Granton Y1 - 2006/11/01 UR - http://www.jrheum.org/content/33/11/2347.abstract N2 - OBJECTIVE: 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. ER -