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Immune-Related Comorbidities in Pediatric Familial Mediterranean Fever: A Hidden Burden beyond Autoinflammation

Fatih Eren1, Sefika Ilknur Kokcu Karadag2, Alisan Yıldıran3

  • 1Department of Pediatrics, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey.

International Archives of Allergy and Immunology
|September 2, 2025
PubMed

Insights

A significant portion of pediatric Familial Mediterranean Fever patients exhibit immune-related comorbidities, often presenting with atypical symptoms. Early immunologic assessment is crucial for identifying these cases and improving treatment outcomes.

Area of Science:

  • Pediatric Rheumatology
  • Clinical Immunology
  • Genetics

Background:

  • Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
  • Immune-related comorbidities in pediatric FMF are not well-characterized.
  • Understanding these comorbidities can refine diagnostic and therapeutic approaches.

Purpose of the Study:

  • To determine the frequency of immune-related comorbidities in children with FMF.
  • To analyze the clinical and immunologic characteristics of these patients.
  • To compare patients with and without comorbidities.

Main Methods:

  • A cohort study of 132 pediatric FMF patients at a tertiary care center.
  • Stratification into groups based on the presence of immune-related comorbidities.
  • Comparative analysis of clinical data, laboratory parameters, MEFV mutations, and treatments.

Main Results:

  • Immune-related comorbidities were found in 37.8% of patients, including inborn errors of immunity.
  • Comorbidity group showed more atypical symptoms (diarrhea, rash) and less classical FMF symptoms.
  • Strong ANA positivity and immunoglobulin deficiencies were linked to comorbidities; intravenous immunoglobulin was used exclusively in this group.

Conclusions:

  • Pediatric FMF patients can have immune dysregulation beyond autoinflammation.
  • Immunologic assessment is vital for FMF patients with atypical symptoms or poor colchicine response.
  • Early identification and intervention can enhance outcomes in this subgroup.
Abstract

Keywords:
AutoinflammationComorbiditiesFamilial Mediterranean feverImmune systemPediatric patients

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