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Chest pain in children with familial Mediterranean fever

Emine Nur Sunar-Yayla1, Pelin Esmeray Şenol1, Deniz Gezgin Yıldırım1

  • 1Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Türkiye.

The Turkish Journal of Pediatrics
|January 11, 2024
PubMed
Abstract

Insights

Familial Mediterranean fever (FMF) patients with chest pain experience earlier symptom onset and increased disease severity. The M694V homozygous mutation is more prevalent in these individuals, indicating distinct clinical and genetic profiles.

Area of Science:

  • Genetics
  • Immunology
  • Pediatrics

Background:

  • Familial Mediterranean fever (FMF) is an autoinflammatory disease.
  • Common symptoms include fever, abdominal pain, chest pain, and arthritis.
  • FMF is inherited in an autosomal recessive pattern.

Purpose of the Study:

  • To compare pediatric FMF patients with and without chest pain.
  • To identify clinical, laboratory, and genetic differences.
  • To understand the impact of chest pain on FMF presentation.

Main Methods:

  • Retrospective analysis of 1134 FMF patients (2006-2022).
  • Patients divided into groups with and without recurrent chest pain.
  • Comparison of demographic, clinical, treatment, and MEFV gene data.

Main Results:

  • 14.3% of patients experienced recurrent chest pain.
  • Chest pain group had younger age of onset, higher FMF family history, and increased attack frequency/duration.
  • Higher Pras disease activity scores, colchicine/anti-IL-1 treatment doses, and M694V homozygous mutations were observed in the chest pain group.

Conclusions:

  • Recurrent chest pain in FMF is associated with earlier onset and greater disease severity.
  • Patients with chest pain are more likely to have a family history of FMF.
  • Homozygous M694V mutation is more common in FMF patients with chest pain.
Keywords:
chest painfamilial Mediterranean feverpleurisypyrin

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