PT  - JOURNAL ARTICLE
AU  - Sindhu R Johnson
AU  - Demetris Patsios
AU  - David M Hwang
AU  - John T Granton
TI  - Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.
DP  - 2006 Nov 01
TA  - The Journal of Rheumatology
PG  - 2347--2350
VI  - 33
IP  - 11
4099  - http://www.jrheum.org/content/33/11/2347.short
4100  - http://www.jrheum.org/content/33/11/2347.full
SO  - J Rheumatol2006 Nov 01; 33
AB  - 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.