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/presurned 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.