Open AccessPulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma: A case of pulmonary capillary hemangiomatosis
Author(s) -
Gugnani Manish K.,
Pierson Chris,
Vanderheide Richard,
Girgis Reda E.
Publication year - 2000
Publication title -
arthritis & rheumatism
Language(s) - English
Resource type - Journals
eISSN - 1529-0131
pISSN - 0004-3591
DOI - 10.1002/1529-0131(200003)43:3<699::aid-anr28>3.0.co;2-o
Subject(s) - medicine , prostacyclin , pulmonary edema , scleroderma (fungus) , pulmonary hypertension , edema , autopsy , vasodilation , cardiology , lung , pathology , inoculation
Continuous intravenous infusion of prostacyclin is an effective treatment for primary pulmonary hypertension (PPH), and has recently been shown to be of benefit in PH associated with scleroderma (SSc). Pulmonary capillary hemangiomatosis (PCH) is a rare cause of PPH. Prostacyclin therapy has been complicated by pulmonary edema in cases of PCH. We describe a case of PH associated 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 diagnosis. This is the first reported case of PCH in SSc.