Biyajima S, Osada T, Daidoji H, Hisaoka T, Sakakibara Y, Tajima J, Nakazawa M, Kuroda H, Kobayashi S
Department of Medicine, Koutou Hospital, Tokyo.
Intern Med. 1994 Dec;33(12):768-72. doi: 10.2169/internalmedicine.33.768.
A 57-year-old female patient with Sjögren's syndrome was complicated with pulmonary hypertension (PH) and antiphospholipid antibody (aPL). She had a history of fetal losses, deep vein thrombosis and chronic thyroiditis. On admission, severe pulmonary hypertension, thrombocytopenia, lupus anticoagulant and a decreased level of protein C were found. Pulmonary artery perfusion scintigram revealed multiple defects. She died suddenly despite an intensive therapy. Intimal proliferation with angiomatoid lesions in small pulmonary arteries was observed by autopsy. Since a close relationship between PH and aPL in connective tissue disease is found, it is important to carefully analyze the antiphospholipid antibodies in patients with PH.
