Relapsing catastrophic antiphospholipid syndrome: report of three cases.
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.
Asherson
Ronald A
RA
Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg, South Africa. ashron@icon.co.za
Espinosa
Gerard
G
Menahem
Solomon
S
Yinh
Janet
J
Bucciarelli
Silvia
S
Bosch
Xavier
X
Cervera
Ricard
R
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
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab