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.
Subject(s)
Adult , Female , Humans , Acute Kidney Injury , Adrenal Glands , Antibodies, Antiphospholipid , Antiphospholipid Syndrome , Biopsy , Connective Tissue Diseases , Cyclophosphamide , Disseminated Intravascular Coagulation , Gastrointestinal Tract , Heart Failure , Heparin , Kidney , Lung , Oral Ulcer , Plasmapheresis , Respiratory Distress Syndrome , Seizures , Thrombotic MicroangiopathiesABSTRACT
A 45-year-old woman was admitted to our hospital because of a high fever, dyspnea, and myalgia. At the time of admission, a diagnosis of systemic lupus erythematosus(SLE) was made by fulfilling four of the 1982 American College of Rheumatology criteria with increasing levels of anti-nuclear antibody titer(speckled pattern). Prednisolone given orally in an initial dosage of 30mg/day was not effective and she was expired by respiratory failure due to disseminated intravascular coagulation. A diagnosis of hemophagocytic syndrome was made because of the increased number of unusual hemophagocytic cells in the bone marrow. High levels of serum ferritin which are known to reflect macrophage activition, supported the diagnosis of hemophagocytic syndrome. Hemophagocytic syndrome is characterized by activated phagocytosis presumably induced by hypersecretion of cytokines. Malignant lymphoma and infection are the two representative diseases which may cause hemophagocytic syndrome. Recently several acute lupus hemophagocytic syndromes were reported in patients with SLE. Here we report a case of acute lupus hemophagocytic syndrome observed in a patient with SLE with brief review of literatures.