Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma - A case of pulmonary capillary hemangiomatosis

Continuous intravenous infusion of prostacyclin is an effective treatment f or primary pulmonary hypertension (PPH), and has recently been shown to be of benefit in PH associated with scleroderma (SSc). Pulmonary capillary hem angiomatosis (PCH) is a rare cause of PPH, Prostacyclin therapy has been co mplicated by pulmonary edema in cases of PCH, We describe a case of PH asso ciated with limited SSc, where treatment with prostacyclin was complicated by pulmonary edema. Autopsy revealed PCH as the pathologic basis for the PH . There were no clinical features of PCH prior to initiation of vasodilator therapy, illustrating the potential difficulty in establishing the diagnos is. This is the first reported case of PCH in SSc.