A Case of Catastrophic Primary Antiphospholipid Syndrome.
- Author:
Joon Hyung DOH
1
;
Jung Yoon CHOE
;
Jae Hoon KIM
;
Chang Soo KIM
;
Dong Ho OH
;
Byung Reul CHOI
;
Jae Kean RYU
;
Dae Sung HYUN
;
Ho Gak KIM
;
Yong Jin KIM
;
Sang Gyung KIM
Author Information
1. Department of Internal Medicine, Pathology, School of Medicine, Catholic University of Taegu-Hyosung, Taegu, Korea.
- Publication Type:Case Report
- Keywords:
Catastrophic antiphospholipid syndrome;
Microangiopathic nephropathy
- MeSH:
Acute Kidney Injury;
Adrenal Glands;
Adult;
Antibodies, Antiphospholipid;
Antiphospholipid Syndrome*;
Biopsy;
Connective Tissue Diseases;
Cyclophosphamide;
Disseminated Intravascular Coagulation;
Female;
Gastrointestinal Tract;
Heart Failure;
Heparin;
Humans;
Kidney;
Lung;
Oral Ulcer;
Plasmapheresis;
Respiratory Distress Syndrome, Adult;
Seizures;
Thrombotic Microangiopathies
- From:Korean Journal of Medicine
1999;56(2):240-246
- CountryRepublic of Korea
- Language:Korean
-
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.
