A Case of Catastrophic Primary Antiphospholipid Syndrome / 대한내과학회지

ABSTRACT

Catastrophic antiphospholipid syndrome is a rare clinical syndrome characterized by acute multi-organ failure occurring in patients with antiphospholipid antibodies. It is associated with involvement of several end-organs particularly kidneys, lungs, gastrointestinal tracts and adrenal glands and presents catastrophic clinical pictures such as acute renal failure with thrombotic microangiopathy, myocardial failure, adult respiratory distress syndrome, convulsion and disseminated intravascular coagulation. Conventional treatments(e.g. intravenous heparin, steroid, immunosuppressants) were not effective, while plasmapheresis seems to be a useful therapy. We experienced a case of catastrophic primary antiphospholipid syndrome in 41-year-old woman proved by renal biopsy and immuno-serological tests. She developed acute renal failure, multiple esophageal and oral ulcers, adult respiratory distress syndrome, abnormal elevation of hepatic and pancreatic enzymes and signs of disseminated intravascular coagulation. Evidences of any other connective tissue diseases were not found. Renal biopsy revealed features of thrombotic microangiopathic nephropathy and serum antiphospholipid antibody level was elevated(34GPL). In spite of steroid, cyclophosphamide and supportive therapies, her respiratory distress was not improved.