Article ; Online: Pediatric antiphospholipid syndrome: clinical features and therapeutic interventions in a single center retrospective case series.
Pediatric rheumatology online journal
2022 Volume 20, Issue 1, Page(s) 17
Abstract: Background/purpose: Pediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was ... ...
| Abstract | Background/purpose: Pediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was to review a large institution's experience to better understand the characteristics of children with APS. Methods: We conducted a retrospective review of pediatric APS at a tertiary referral center. The electronic medical record system was queried from 2000 through 2019, and 21 cases were included based on meeting the revised Sapporo Classification criteria by age 18 or younger. Comparisons between primary and secondary APS patients were made with two-tailed t-tests. Results: Twenty-one patients were included with a median age at diagnosis of 16 years and median follow-up of 5.8 years. Secondary APS was slightly more common than primary APS (11 vs. 10 cases) and was primarily diagnosed in the context of systemic lupus erythematosus. Two thirds of patients (67%) also had "non-criteria" manifestations of APS including thrombocytopenia, autoimmune hemolytic anemia, and livedo reticularis/racemosa. Almost half of patients (43%) had recurrent thrombosis, typically when patients were subtherapeutic or non-adherent with anticoagulation. Damage Index in Patients with Thrombotic APS (DIAPS) scores indicated a chronic burden of disease in both primary and secondary APS patients. Conclusion: This case series of pediatric APS provides important context regarding disease phenotypes displayed by children with APS. High prevalence of non-criteria clinical manifestations highlights the need to consider these characteristics when developing pediatric-specific classification criteria and when considering this relatively rare diagnosis in pediatric practice. |
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| MeSH term(s) | Adolescent ; Antibodies, Antiphospholipid/blood ; Anticoagulants/therapeutic use ; Antiphospholipid Syndrome/diagnosis ; Antiphospholipid Syndrome/immunology ; Antiphospholipid Syndrome/physiopathology ; Antiphospholipid Syndrome/therapy ; Cost of Illness ; Female ; Humans ; Immunomodulating Agents/classification ; Immunomodulating Agents/therapeutic use ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/immunology ; Male ; Patient Compliance ; Platelet Aggregation Inhibitors/therapeutic use ; Retrospective Studies ; Severity of Illness Index ; Symptom Assessment/methods ; Symptom Assessment/statistics & numerical data ; Tertiary Care Centers/statistics & numerical data ; Thrombosis/blood ; Thrombosis/etiology ; Thrombosis/therapy ; Treatment Outcome |
| Chemical Substances | Antibodies, Antiphospholipid ; Anticoagulants ; Immunomodulating Agents ; Platelet Aggregation Inhibitors |
| Language | English |
| Publishing date | 2022-02-23 |
| Publishing country | England |
| Document type | Journal Article |
| ZDB-ID | 2279468-2 |
| ISSN | 1546-0096 ; 1546-0096 |
| ISSN (online) | 1546-0096 |
| ISSN | 1546-0096 |
| DOI | 10.1186/s12969-022-00677-8 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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