The challenges in diagnosing pediatric primary antiphospholipid syndrome

Demir S., Keskin A., Sag E., Akca U. K., Atalay E., Kasap Cüceoğlu M., ...More

LUPUS, vol.31, no.10, pp.1269-1275, 2022 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 31 Issue: 10
  • Publication Date: 2022
  • Doi Number: 10.1177/09612033221108853
  • Journal Name: LUPUS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, EMBASE, MEDLINE
  • Page Numbers: pp.1269-1275
  • Keywords: Pediatric Antiphospholipid Syndrome, Primary Antiphospholipid Syndrome, Nonthrombotic Manifestations in Antiphospholipid Syndrome, INTERNATIONAL CONSENSUS STATEMENT, SYSTEMIC-LUPUS-ERYTHEMATOSUS, CLASSIFICATION CRITERIA, ANTIBODY PROFILES, CHOREA
  • Eskisehir Osmangazi University Affiliated: No


Pediatric primary antiphospholipid syndrome (APS) is a very rare disease with significant distinctions from the APS in adults. Herein, we present our experience in the diagnosis and treatment of six pediatric primary APS patients, who met the updated Sapporo criteria for the APS diagnosis. One of them was also diagnosed as having probable catastrophic APS (CAPS) due to the involvement of three different organ systems simultaneously. Besides vascular involvement, four patients had thrombocytopenia, one had psychiatric disorder, and one had chorea and valvular heart disease. All patients received immunosuppressive treatment along with long-term anticoagulation therapy. Specific neurologic and hematologic manifestations that are not part of the classification criteria can be seen in children with primary APS. Therefore, using the adult criteria for diagnosing pediatric APS may result in missed or delayed diagnoses in children.