title: Treatment of catastrophic antiphospholipid syndrome.
abstract: OBJECTIVE
  Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS.
  
  
  RESULTS
  Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses. Current guidelines also continue to support the role of anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab, warrant further study.
  
  
  CONCLUSIONS
  Attention to associated diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical at the time of diagnosis. All patients should be treated with anticoagulants, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should be considered. In refractory or relapsing cases, new therapies, such as rituximab and possibly eculizumab, may be options, but need further study.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.
authors.forename: Nayef M
authors.initials: NM
authors.lastname: Kazzaz
authors.forename: W Joseph
authors.initials: WJ
authors.lastname: McCune
authors.forename: Jason S
authors.initials: JS
authors.lastname: Knight
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000628:therapy*; D002388:Catastrophic Illness / Q000628:therapy*; D019468:Disease Management; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D010951:Plasma Exchange / Q000379:methods*; D017410:Practice Guidelines as Topic; D012008:Recurrence
chemical_list: D000925:Anticoagulants; D005938:Glucocorticoids