Pediatric Catastrophic Antiphospholipid Syndrome: Case Study and Literature Review

Abstract

nancy loss in the presence of antiphospholipid antibodies (aPL) including lupus anticoagulant (LAC), anticardiolipin (aCL) or anti-β2-glycoprotein-1 (anti-β2GP-1) [1]. Primary APS occurs spontane- AbstractBackground: Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially fatal variant of antiphospholipid syndrome associated with small vessel thromboses and multi-organ failure. Methods: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.Results: 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.Conclusions: 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.

What Have We Learnt About the Treatment of Juvenile-Onset Systemic Lupus Erythematous Since Development of the SHARE Recommendations 2012?

“…A case series of 21 patients with pediatric CAPS (from 1990 to 2013) was found, which suggested that immunosuppression with corticosteroids or rituximab may confer survival benefit. In this study, none of the patients who received rituximab died, however, the odds ratio for survival crossed 1 and was not statistically significant, potentially likely relating to the small sample size ( 74 ). Case reports have also suggested ecluzimab may be beneficial in treatment of CAPS in adults ( 75 , 76 ) however this has not yet been assessed in children.…”

Catastrophic Antiphospholipid Syndrome: A Review

“…Venous thrombosis was the most common form of peripheral vascular involvement. From 1992 to 2014, Defreitas et al [47,57] analyzed and summarized 21 published cases of pediatric CAPS. The age ranged from 10.5 to 4.8 years on average.…”

“…Anticoagulant therapy is challenging in kids. This is due to the fact that children's hemostasis, pharmacodynamic, and pharmacokinetic responses to antithrombotics differ from those of adults [57,58]. As a result, dosing, monitoring, and drug interactions in children are more difficult.…”