Comparison of Clinical Features Between Early-onset (≤2 Years) and Later-onset Familial Mediterranean Fever


Özçelik E., Uğur Es Y., Erdem Torun Ş., Öztürk D., Yoğun S. N., Polat M. C., ...Daha Fazla

Journal of Clinical Rheumatology, cilt.32, sa.5, ss.157-162, 2026 (SCI-Expanded, Scopus)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 32 Sayı: 5
  • Basım Tarihi: 2026
  • Doi Numarası: 10.1097/rhu.0000000000002345
  • Dergi Adı: Journal of Clinical Rheumatology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CINAHL, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.157-162
  • Anahtar Kelimeler: age of onset, colchicine, familial Mediterranean fever, pediatrics
  • Ankara Yıldırım Beyazıt Üniversitesi Adresli: Evet

Özet

Objective: – The objective of this study was to investigate differences between familial Mediterranean fever (FMF) patients with symptom onset at aged 2 years or younger and those with later onset, with a focus on clinical presentation, disease course, and treatment outcomes. Materials and Methods: – The medical records of FMF patients aged 0 to 18 years who were followed in the Pediatric Rheumatology Department between 2013 and 2024 were reviewed. Patients with symptom onset at aged 2 years or younger were classified as having early-onset, and patients with missing data or <6 months of follow-up were excluded. Results: – A total of 1255 patients with FMF were divided into 2 groups according to the age at symptom onset: ≤2 years (early-onset FMF, n = 346) and >2 years (later-onset FMF, n = 909). Early-onset FMF patients exhibited longer diagnostic delays, higher attack frequency, and more frequent fever attacks compared with later-onset patients (p < 0.05). Disease severity and the prevalence of colchicine-resistant FMF were higher in the early-onset group (p = 0.021 and p = 0.002). M694V homozygosity was more common among early-onset FMF patients (29.9% vs. 22.1%, p = 0.005). In multivariate analysis, a family history of colchicine-resistant FMF (OR = 2.64, 95% CI: 1.48-4.71) and fever (OR = 3.05, 95% CI: 2.12-4.40) were identified as independent predictors of early disease onset FMF. Conclusion: – Early-onset FMF is associated with a more severe clinical presentation, including higher disease severity, increased colchicine resistance, and a greater frequency of M694V homozygosity. A family history of colchicine-resistant FMF is an independent predictor of early onset, highlighting the importance of detailed family history assessment in clinical practice.