Background/Purpose: A systematic review of case reports to summarize existing evidence in the literature regarding the association of APS and infection during childhood. Our aims were to identify all possible presumed infections that may predispose to elevation of aPL antibodies, and related clinical events.

Methods: Medline, EMBASE, Web of Science, PubMed ePubs, and the Cochrane Central Register of Controlled Trials (CENTRAL) were searched with no restriction through June 2016. References cited in the included articles were searched manually. We included case reports describing children who developed elevated aPL antibodies after a prior infection. One positive laboratory test for anticardiolipin, lupus anticoagulant, or anti-β2-GPI antibodies after a previous diagnosis of infection was required for inclusion. We extracted data on the infectious agent identified, pre-existing conditions, aPL antibody profile, clinical manifestations, treatment required, and reported outcome. Patients with systemic lupus were excluded.

Results: A total of 2,740 unique citations were identified through the databases and hand-searched bibliographies. Of these, 72 publications met inclusion criteria, reporting on 87 cases. The age of the cases ranged from 6 months to 18 years; 48 children (55.2%) were female. Cases were classified into four groups according to the clinical presentation reported following infection: 1) patients fulfilling criteria for diagnosis of APS (13.8%) including three cases with catastrophic antiphospholipid syndrome (CAPS), 2) patients with transient thromboembolic events, not fulfilling APS criteria (31.0%), 3) patients developing hemorrhage rather than thrombosis (31.0%), and 4) patients with elevated aPL antibodies but no clinical manifestations (24.2%). Viral infection was the most frequent preceding infections across all groups (56.3%), followed by bacterial infection (26.0%). Ten different viral and bacterial agents were reported in group 1, with Escherichia coli, Pseudomonas aeruginosa, and Parvovirus B19 recognized in the three cases who developed CAPS. Varicella Zoster and Mycoplasma Pneumonia were mainly identified in group 2, Adenovirus in group 3, and both Adenovirus and Parvovirus B19 were equally reported in group 4. Infection was the sole precipitating factor in 85.1% of the reported cases; 8.1% had pre-existing autoimmune diseases such as juvenile idiopathic arthritis, or celiac disease, where Parvovirus B19 was the predominant infection. Thirty nine (44.8%) cases developed thromboembolic events, predominantly hematologic, followed by skin manifestations, peripheral thrombosis, and stroke. Splenic infarction and other non-typical presentations were also reported. Positive lupus anticoagulant was most frequently reported in (81.7%). Anticoagulation or antiplatelet therapy were required for the majority of cases in groups 1 and 2. While complete recovery was achieved in the majority of cases, 37.5% of patients in group 1 reported persistent APS, and 25.0% died.

Conclusion: Development of aPL antibodies, APS and CAPS can occur in children after an infection. Further studies are needed to determine the risk and long-term outcomes of aPL-related events following infection.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/antiphospholipid-antibodies-and-related-clinical-events-following-infection-in-children-a-systematic-review-of-case-reports/