Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases · 2026
Comparison of Clinical Features Between Early-onset (≤2 Years) and Later-onset Familial Mediterranean Fever.
СтатьяСравнительное исследованиеНазвание и аннотация: Перевод в очереди
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Özçelik Emine, Uğur Es Yasemin, Erdem Torun Şeyma, Öztürk Didem, Yoğun Sultan Nilay, Polat Merve Cansu, Işıklar Ekici Mehveş, Çelikel Elif, Ekici Tekin Zahide, Ertem Şeyma, Karagöl Cüneyt, Çelikel Acar Banu
Аннотация
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.
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.