Validity of the 2023 ACR/EULAR APS classification criteria for paediatric primary APS: inputs from a systematic literature review.
Stefano Romano, Edoardo Marrani, Valerio Maniscalco et al.
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In 30 seconds
This systematic literature review and individual participant data-style random-effects meta-analysis assessed the 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria in children with primary APS. It included 148 patients identified across 118 studies. The 2023 criteria had a pooled sensitivity of 83.1% (95% CI: 76.2-88.3), compared with 91.2% (95% CI: 85.4-95.2) for the 2006 Sapporo criteria. Most children who did not meet the 2023 criteria failed to satisfy laboratory domains, indicating that adult-derived criteria may not fully capture paediatric disease.
Key findings
- Thrombotic events were reported in 93.2% of patients, and non-thrombotic manifestations in 56.8%.
- Thrombocytopenia was the most frequent extra-thrombotic manifestation, occurring in 29%.
- Sensitivity was 83.1% (95% CI: 76.2-88.3) for the 2023 criteria and 91.2% (95% CI: 85.4-95.2) for the 2006 Sapporo criteria.
- Most patients not classified by the 2023 criteria failed to meet laboratory domains.
Why it matters
Classification criteria shape how patients are identified and grouped in research. The lower sensitivity observed with the 2023 criteria suggests that some children with primary APS may be missed, particularly because of laboratory thresholds and clinical features relevant to paediatric disease. This highlights the incomplete overlap between paediatric and adult-onset APS and the importance of evaluating criteria in the population where they are used.
What to keep in mind
The evidence was assembled from published studies rather than a prospective paediatric cohort, and the analysis included only 148 patients across 118 studies.