Autoinflammatory & rare systemic diseases

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation

Pediatr Rheumatol Online JPublished Registry-based retrospective cohort study

Authors' conclusion

Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group.

Study context

Population
Patients with recurrent fevers without molecular diagnosis (PFAPA, syndrome of undifferentiated recurrent fever (SURF) and uSAID) in European AID registries3 registries
Exposure
PFAPA vs SURF vs uSAID
Outcome
Evolution of symptoms in recurrent fevers without molecular diagnosis
Methodological detailsmultiregistry (3 European AID registries), retrospective application of classification criteria
  • Multiregistry (3 European AID registries)
  • Retrospective application of classification criteria

Main finding

As reported in the abstract

  • Clinical presentation of PFAPA is variable; some patients did not fit conventional PFAPA criteria
  • Some patients met criteria for neither PFAPA nor SURF
  • Some patients exhibited symptoms characteristic of both PFAPA and SURF/undefined autoinflammatory diseases (uAID)

Observational data: associations, not effects.

Original publication

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation. Pediatr Rheumatol Online J. Published 17 May 2024. doi:10.1186/s12969-024-00987-z

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