TY - JOUR
T1 - Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma
T2 - A case of pulmonary capillary hemangiomatosis
AU - Gugnani, Manish K.
AU - Pierson, Chris
AU - Vanderheide, Richard
AU - Girgis, Reda E.
PY - 2000/3
Y1 - 2000/3
N2 - 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.
AB - 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.
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U2 - 10.1002/1529-0131(200003)43:3<699::AID-ANR28>3.0.CO;2-O
DO - 10.1002/1529-0131(200003)43:3<699::AID-ANR28>3.0.CO;2-O
M3 - Article
C2 - 10728766
AN - SCOPUS:0034048133
SN - 0004-3591
VL - 43
SP - 699
EP - 703
JO - Arthritis and rheumatism
JF - Arthritis and rheumatism
IS - 3
ER -