Document Detail


Catastrophic antiphospholipid syndrome.
MedLine Citation:
PMID:  19694227     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Antiphospholipid syndrome (APS) is an autoimmune disease with clinical manifestations of arterial and venous thrombosis, concomitant fetal loss and the presence of antiphospholipid antibodies (APLA). This report focuses on the challenges of optimal treatment involving plasma exchange and intravenous human immunoglobulin infusions that is administered in patients with catastrophic APS (CAPS). CAPS is a rare variant of APS defined as acute failure of at least three tissues, organs or systems caused predominantly by small vessel thrombosis confirmed by histopathologic evidence. CAPS develops rapidly and leads to death in 50% of cases. We present the case of a 39-year-old male patient with APS with worsening renal function. Positive lupus anticoagulant, markedly high concentrations of anticardiolipin and anti-beta 2-glikoprotein I antibodies have been observed. According to the criteria introduced by Asherson, a catastrophic form of APS was diagnosed and the patient had been treated with low-molecular-weight heparin, glucocorticosteroids, and plasmapheresis. In order to maintain clinical improvement, the patient was given human immunoglobulins i.v. (1 g/kg body weight). After the procedure, gradual clinical improvement was observed and renal function remained stable (serum creatinine level of 1.5 mg/dl).
Authors:
Agnieszka Furma?czyk; Ewa Komuda-Leszek; Wioletta Gadomska; Jerzy Windyga; Magdalena Durlik
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Polskie Archiwum Medycyny Wewn?trznej     Volume:  119     ISSN:  0032-3772     ISO Abbreviation:  Pol. Arch. Med. Wewn.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-08-21     Completed Date:  2009-09-17     Revised Date:  2010-04-01    
Medline Journal Info:
Nlm Unique ID:  0401225     Medline TA:  Pol Arch Med Wewn     Country:  Poland    
Other Details:
Languages:  eng     Pagination:  427-30     Citation Subset:  IM    
Affiliation:
Department of Transplantation Medicine and Nephrology, Transplantation Institute, Medical University of Warsaw, Warszawa, Poland. afurmanczyk@gmail.com
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MeSH Terms
Descriptor/Qualifier:
Adult
Antibodies, Anticardiolipin / blood
Antiphospholipid Syndrome / blood,  complications,  diagnosis*,  therapy*
Catastrophic Illness
Combined Modality Therapy
Glucocorticoids / administration & dosage*
Heparin, Low-Molecular-Weight / administration & dosage*
Humans
Kidney Failure, Acute / etiology,  therapy
Lupus Coagulation Inhibitor / blood
Male
Plasmapheresis / methods*
Treatment Outcome
beta 2-Glycoprotein I / blood
Chemical
Reg. No./Substance:
0/Antibodies, Anticardiolipin; 0/Glucocorticoids; 0/Heparin, Low-Molecular-Weight; 0/Lupus Coagulation Inhibitor; 0/beta 2-Glycoprotein I

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