An autopsied case of systemic lupus erythematosus with pulmonary hypertension is 
reported. A 29-year-old woman with a seven-year history of polyarthralgia, 
butterfly rash, nephrotic syndrome and Raynaud's phenomenon was admitted because 
of progressive dyspnea on exertion. Tests for antinuclear antibody, 
anti-cardiolipin antibody and lupus anticoagulant were positive. 
Echocardiographic examination revealed right ventricular hypertrophy and a 
moderate pericardial effusion. Estimated systolic pulmonary arterial pressure 
was 53 mmHg. Despite treatment with corticosteroids including pulse 
methylprednisolone therapy, lipo-PGE1 and warfarin, she died of progressive 
congestive heart failure. Postmortem examination of the pulmonary vasculature 
revealed findings consistent with plexogenic pulmonary arteriopathy, without 
evidence of vasculitis, fibrinoid necrosis, or thromboemboli.