PULMONARY-HYPERTENSION AND ANTIPHOSPHOLIPID ANTIBODY IN A PATIENT WITH SJOGRENS-SYNDROME

A 57-year-old female patient with Sjogren's syndrome was complicated w ith pulmonary hypertension (PH) and antiphospholipid antibody (aPL). S he had a history of fetal losses, deep vein thrombosis and chronic thy roiditis. On admission, severe pulmonary hypertension. thrombocytopeni a, lupus anticoagulant and a decreased level of protein C were found. Pulmonary artery perfusion scintigram revealed multiple defects. She d ied suddenly despite an intensive therapy. Intimal proliferation with angiomatoid lesions in small pulmonary arteries mas observed by autops y. Since a close relationship between PH and aPL in connective tissue disease is found, it is important to carefully analyze the antiphospho lipid antibodies in patients with PH.