Evans Syndrome Predicts Progression to Antiphospholipid Syndrome and/or Systemic Lupus Erythematosus in Children With Persistent Antiphospholipid Antibodies: A Prospective Cohort Study With Up to 29 Years of Follow‐Up
Main finding · Progression to APS and/or SLEResult — go to the resultHazard ratio 6.21 95% CI 1.46–26.41Authors' conclusion
In children with persistent aPL positivity, Evans syndrome was the hematologic phenotype most strongly associated with progression to APS and/or SLE, whereas isolated thrombocytopenia followed a largely indolent course.
Study context
- Population
- Children (<18 years old) with persistent aPL positivity and hematologic involvement (thrombocytopenia, AIHA or Evans syndrome), tertiary pediatric rheumatology center, 1995–2024, follow-up into adulthood40 evaluable
- Participants
- 42 children
- Exposure
- Hematologic phenotypes at presentation vs isolated thrombocytopenia
- Outcome
- Progression to clinically classifiable APS and/or SLE
Methodological detailssingle-centre (tertiary pediatric rheumatology center), Kaplan-Meier and Cox proportional hazards models, time-dependent analyses
- Single-centre (tertiary pediatric rheumatology center)
- Kaplan-Meier and Cox proportional hazards models
- Time-dependent analyses
Main finding
Progression to APS and/or SLE
Hazard ratio
6.21
95% CI 1.46–26.41
Evans syndromevs Isolated thrombocytopenia
HR 6.21 (95% CI 1.46–26.41)
As reported in the abstract
- Progressed to antiphospholipid syndrome (APS) and/or systemic lupus erythematosus (SLE) (of 40 evaluable)
- 11 (27.5%)
Observational data: associations, not effects.
Further findings
Evans syndrome emerging during follow-up (time-dependent analyses)
- Remained associated with progression
aPL profiles
- LA nearly universal
- Broader high-risk aPL profiles more common among progressors, not statistically significant
Original publication
Evans Syndrome Predicts Progression to Antiphospholipid Syndrome and/or Systemic Lupus Erythematosus in Children With Persistent Antiphospholipid Antibodies: A Prospective Cohort Study With Up to 29 Years of Follow‐Up. Arthritis Rheumatol. Published 16 July 2026. doi:10.
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