PULMONARY-HYPERTENSION IN SYSTEMIC LUPUS-ERYTHEMATOSUS - A REPORT OF AN AUTOPSIED CASE

An autopsied case of systemic lupus erythematosus with pulmonary hyper tension is reported. A 29-year-old woman with a seven-year history of polyarthralgia, butterfly rash, nephrotic syndrome and Raynaud's pheno menon was admitted because of progressive dyspnea on exertion. Tests f or antinuclear antibody, anti-cardiolipin antibody and lupus anticoagu lant were positive. Echocardiographic examination revealed right ventr icular hypertrophy and a moderate pericardial effusion. Estimated syst olic pulmonary arterial pressure was 53 mmHg. Despite treatment with c orticosteroids including pulse methylprednisolone therapy, lipo-PGE1 a nd warfarin, she died of progressive congestive heart failure. Postmor tem examination of the pulmonary vasculature revealed findings consist ent with plexogenic pulmonary arteriopathy, without evidence of vascul itis, fibrinoid necrosis, or thromboemboli.