title: Pulmonary hypertension and antiphospholipid antibody in a patient with Sjögren's syndrome.
abstract: 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.
authors.affiliation: Department of Medicine, Koutou Hospital, Tokyo.
authors.forename: S
authors.initials: S
authors.lastname: Biyajima
authors.forename: T
authors.initials: T
authors.lastname: Osada
authors.forename: H
authors.initials: H
authors.lastname: Daidoji
authors.forename: T
authors.initials: T
authors.lastname: Hisaoka
authors.forename: Y
authors.initials: Y
authors.lastname: Sakakibara
authors.forename: J
authors.initials: J
authors.lastname: Tajima
authors.forename: M
authors.initials: M
authors.lastname: Nakazawa
authors.forename: H
authors.initials: H
authors.lastname: Kuroda
authors.forename: S
authors.initials: S
authors.lastname: Kobayashi
mesh_terms: D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis