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.

Among 42 enrolled children, 40 were evaluable, of whom 11 (27.5%) progressed to antiphospholipid syndrome and/or systemic lupus erythematosus.

In time-dependent analyses, Evans syndrome emerging during follow-up remained associated with progression.

Isolated thrombocytopenia showed the lowest risk, whereas AIHA represented an intermediate state that did not independently predict progression.

Lupus anticoagulant was nearly universal, and broader high-risk aPL profiles were more common among progressors but were not statistically significant.