title: Relapsing catastrophic antiphospholipid syndrome: report of three cases.
abstract: BACKGROUND
  The catastrophic variant of the antiphospholipid syndrome (CAPS), also now known as Asherson's syndrome, is defined as a potential life-threatening variant of the antiphospholipid syndrome, which is characterized by multiple small-vessel thrombosis that can lead to multiorgan failure. Relapses in patients with the CAPS are very uncommon.
  
  
  OBJECTIVE
  To describe the clinical and laboratory features of patients with relapsing episodes of CAPS.
  
  
  METHODS
  Three patients with relapsing CAPS are presented with their clinical and laboratory features.
  
  
  RESULTS
  Seven episodes of CAPS that occurred in the 3 patients reported were analyzed. The median time between the episodes of CAPS was 12.5 months (range, 2.5-48). Precipitating factors were identified in 2 episodes only (Legionella respiratory tract infection and periodontal infection). The most significant manifestations of the episodes were renal involvement (5 episodes), central nervous system and cardiac involvement (4 episodes), and pulmonary and hepatic involvement (3 episodes each). Interestingly, laboratory features of definite microangiopathic hemolytic anemia (MHA) were present in 5 of 7 episodes of relapsing CAPS. The remaining episodes presented with thrombocytopenia, schistocytes, and anemia but data concerning hemolysis and Coombs tests were not reported. Rituximab was used in 2 episodes.
  
  
  CONCLUSIONS
  Relapses occur very infrequently in patients with the CAPS. The presence of MHA is common in these patients, suggesting that an association between MHA and relapses of CAPS could be present and that a "continuum" between various MHAs might exist, as recently suggested.
authors.affiliation: Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa. ashron@icon.co.za
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Asherson
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Solomon
authors.initials: S
authors.lastname: Menahem
authors.forename: Janet
authors.initials: J
authors.lastname: Yinh
authors.forename: Silvia
authors.initials: S
authors.lastname: Bucciarelli
authors.forename: Xavier
authors.initials: X
authors.lastname: Bosch
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
mesh_terms: D054058:Acute Coronary Syndrome / Q000150:complications; D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000188:drug therapy* / Q000628:therapy*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D012008:Recurrence; D051437:Renal Insufficiency / Q000209:etiology; D000069283:Rituximab; D018805:Sepsis / Q000150:complications
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab