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Fevers and the rheumatologist

Prudence Joan Manners1, Robin Guttinger

  • 1School of Paediatrics and Child Health, University of Western Australia, c/- Princess Margaret Hospital for Children, Perth, Australia. pmanners@meddent.uwa.edu.au

Indian Journal of Pediatrics
|October 19, 2010
PubMed

Insights

Pediatric rheumatologists manage fevers caused by inflammatory conditions. This review details seven hereditary recurrent fever syndromes, their diagnosis, and treatments.

Area of Science:

  • Pediatric Rheumatology
  • Genetics
  • Immunology

Background:

  • Fevers in children often stem from infections, malignancies, or inflammatory conditions.
  • Rheumatologists play a key role in managing inflammatory conditions associated with fevers.
  • Seven distinct hereditary recurrent fever syndromes have been identified, each linked to a specific gene mutation.

Purpose of the Study:

  • To provide a comprehensive overview of hereditary recurrent fever syndromes within the context of pediatric rheumatological conditions.
  • To elucidate diagnostic approaches for these rare genetic inflammatory disorders.
  • To outline current treatment strategies for each identified syndrome.

Main Methods:

  • Literature review and synthesis of existing data on hereditary recurrent fever syndromes.
  • Detailed discussion of diagnostic criteria and genetic underpinnings.
  • Analysis of current therapeutic recommendations and management guidelines.

Main Results:

  • Seven hereditary recurrent fever syndromes are detailed: CINCA, FACS, FMF, HIDS, MWS, PAPA, and TRAPS.
  • Each syndrome presents with recurring fevers and is associated with a defined single-gene mutation.
  • Diagnostic pathways and treatment options for each condition are reviewed.

Conclusions:

  • Hereditary recurrent fever syndromes represent a significant group of pediatric inflammatory conditions.
  • Accurate diagnosis relies on recognizing characteristic clinical features and genetic testing.
  • Tailored treatment strategies are crucial for managing these autoinflammatory disorders effectively.

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