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Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially fatal variant of antiphospholipid syndrome associated with small vessel thromboses and multi-organ failure. A case summary with review of the literature since 1992 was performed. Inclusion criteria were patients< 18 years of age who met at least 3 of 4 diagnostic criteria for primary or secondary CAPS. Twenty one patients were identified from case reports or series, ages ranged from. 3 months to 18 years (mean 10.5±4.8 years). Fourteen (66%) were female. The majority (76%) had primary onset; whereas, 5 (24%) were secondary to systemic lupus erythematosus (SLE). There was an infectious trigger in the majority of cases (13= 62%). There were 5 (24%) deaths. Most patients were treated with immunosuppressive regimens including pulse corticosteroids, plasma exchange, cyclophosphamide and/or rituximab. Although not statistically significant, the patients who received immune suppression were 4 times more likely to survive than those who did not. None of the patients who received rituximab died suggesting a survival advantage when used as an adjunctive medication. CAPS should be included in the differential diagnosis of children who present with sudden onset multi-organ failure. Multimodal therapy including plasma exchange and rituximab is potentially life-saving. Appropriate long term treatment regimens need to be developed and rheumatology follow-up is essential.