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Periodic fever syndromes--a clinical overview

Martin Aringer1

  • 1Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria. martin.aringer@akh-wien.ac.at

Acta Medica Austriaca
|July 21, 2004
PubMed

Insights

Familial Mediterranean Fever (FMF) and TNF-Receptor Associated Periodic Syndrome (TRAPS) are rare hereditary periodic fever syndromes that typically manifest in early childhood. This review focuses on these two conditions, which can present later in life.

Area of Science:

  • Genetics and immunology of autoinflammatory diseases.

Background:

  • Hereditary periodic fever syndromes (HPFS) usually begin in infancy.
  • Familial Mediterranean Fever (FMF) and TNF-Receptor Associated Periodic Syndrome (TRAPS) are exceptions, often presenting later in childhood or adolescence.

Purpose of the Study:

  • To review the key features of Familial Mediterranean Fever (FMF) and TNF-Receptor Associated Periodic Syndrome (TRAPS).
  • To highlight FMF and TRAPS as distinct HPFS with later onset.

Main Methods:

  • Literature review focusing on FMF and TRAPS.
  • Analysis of clinical presentation and epidemiological data for FMF and TRAPS.

Main Results:

  • FMF and TRAPS are the primary HPFS with onset beyond the first year of life.
  • FMF incidence is increasing in Central Europe due to migration.
  • TRAPS is a considerably rarer condition.

Conclusions:

  • FMF and TRAPS represent important late-onset hereditary periodic fever syndromes.
  • Understanding these conditions is crucial for timely diagnosis and management, particularly with changing demographics.

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