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Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects

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Abstract

Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.

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  1. Aline Garcia Islabão and Vitor Cavalcanti Trindade contributed equally to this paper.

Authors and Affiliations

  1. Pediatric Rheumatology Unit, Hospital da Criança de Brasília Jose Alencar, Brasília, DF, Brazil

    Aline Garcia Islabão

  2. Programa de Pós-graduação em Ciências Médicas, Faculdade de Medicina, Universidade de Brasília, Brasília, DF, Brazil

    Aline Garcia Islabão & Licia Maria Henrique da Mota

  3. Faculdade de Medicina, Children and Adolescent Institute, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Av. Dr. Enéas Carvalho de Aguiar, 647-Cerqueira César, São Paulo, SP, 05403-000, Brazil

    Vitor Cavalcanti Trindade & Clovis Artur Silva

  4. Rheumatology Unit, Hospital Universitário de Brasília, Universidade de Brasília, Brasília, Brazil

    Licia Maria Henrique da Mota

  5. Rheumatology Division, Faculdade de Medicina, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, São Paulo, Brazil

    Danieli Castro Oliveira Andrade & Clovis Artur Silva

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  1. Aline Garcia Islabão
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Our study was supported by grants to CASE from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 304984/2020-5), Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP 2015/03756-4), and Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd).

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Aline Garcia Islabão, Vitor Cavalcanti Trindade, Licia Maria Henrique da Mota, Danieli Castro Oliveira Andrade, and Clovis Artur Silva have no conflicts of interest that are directly relevant to the content of this article.

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Islabão, A.G., Trindade, V.C., da Mota, L.M.H. et al. Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects. Pediatr Drugs 24, 13–27 (2022). https://doi.org/10.1007/s40272-021-00484-w

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