Antiphospholipid syndrome in the paediatric population: performance of ACR-EULAR classification criteria in a controlled cohort
Authors' conclusion
The superior sensitivity and likelihood ratio of the 2023 ACR-EULAR criteria suggest they may be a valuable tool for the classification of paediatric APS
Study context
- Population
- Patients ≤18 years with antiphospholipid syndrome (APS) or aPL carriers (controls), Sheba Medical Center, 2011–2024; mean age 16 years, 62% female29 APS, 40 aPL carriers
- Participants
- 69 patients
- Comparison
- 2023 ACR-EULAR APS classification criteria vs 2006 revised Sapporo criteria
- Outcome
- Diagnostic performance of the ACR-EULAR APS criteria vs traditional criteria (APS diagnosis by expert opinion, validated by two independent experts)
Methodological detailsretrospective, single-centre
- Retrospective
- Single-centre
Performance
As reported in the abstract
Diagnostic performance (likelihood ratios) for paediatric APS
| Test or model | Positive likelihood ratio | Negative likelihood ratio |
|---|---|---|
| 2023 ACR/EULAR criteria | 18.23 | 0.036 |
| 2006 Sapporo criteria | 16.43 | 0.136 |
Positive likelihood ratio
2023 ACR/EULAR criteria18.23
2006 Sapporo criteria16.43
Negative likelihood ratio
2023 ACR/EULAR criteria0.036
2006 Sapporo criteria0.136
Further findings
Predictors of APS (APS patients vs aPL carriers)
| OR (95% CI) | ||
|---|---|---|
| Unprovoked venous thrombosis | 1.55 (1.02–2.36) | |
| aGAPSS | 1.053 (1.031–1.076) | |
| Platelet number | 0.999 (0.998–1) | |
| 0.512Odds ratio (log scale) |
| Outcome | Values |
|---|---|
| Catastrophic APS | APS 10.3% |
| Mortality | APS 6.7% · aPL carriers 0% |
Family history of APS (APS more common with family history)
- Family history of APS, 37.5% vs 17.5%
- P = 0.056
Non-thrombotic manifestations (heart valve disease, livedo reticularis, diffuse alveolar haemorrhage)
- Only present in the APS group
Original publication
Antiphospholipid syndrome in the paediatric population: performance of ACR-EULAR classification criteria in a controlled cohort. Lupus Sci Med. Published 29 June 2026. doi:10.
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