Pulmonary Hypertension and Antiphospholipid Antibody in a Patient with Sjoegren's Syndrome.

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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.(Internal Medicine 33: 768-772, 1994)