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Familial Mediterranean Fever -- an increasingly important childhood disease in Sweden

P Wekell1, V Friman, B Balci-Peynircioglu

  • 1Department of Pediatrics, University of Gothenburg, Sweden.

Acta Paediatrica (Oslo, Norway : 1992)
|December 1, 2012
PubMed
Abstract

Insights

Familial Mediterranean Fever (FMF) in western Sweden primarily affects children, with prevalence similar to origin countries. Increased awareness is crucial to shorten the diagnostic timeline for this autoinflammatory disease.

Area of Science:

  • Genetics and Immunology
  • Rheumatology
  • Epidemiology

Background:

  • Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
  • Characterizing FMF in diverse populations is essential for understanding its clinical spectrum.

Purpose of the Study:

  • To characterize Familial Mediterranean Fever (FMF) in western Sweden.
  • Focus on genotype, clinical presentation, prevalence, age of onset, and time to diagnosis.

Main Methods:

  • Retrospective analysis of FMF patient records from five hospitals in Western Sweden.
  • Utilized population data on immigration from Statistics Sweden.
  • Screened patients for common FMF mutations.

Main Results:

  • Identified 37 FMF patients by 2008; prevalence varied among immigrant groups.
  • Median age at onset was 4 years, diagnosis at 10 years, with a median diagnostic delay of 4 years.
  • 75% of screened patients had homozygous or compound heterozygous mutations; common symptoms included fever (100%) and peritonitis (92%).

Conclusions:

  • FMF predominantly presents in childhood.
  • Prevalence in immigrants aligns with their countries of origin.
  • Enhanced disease awareness is needed to reduce diagnostic delays.

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