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Is age associated with disease severity and compliance to treatment in children with familial Mediterranean fever?

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Abstract

Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease in the world. The disease characteristics may vary in different age groups. In this study, we aimed to compare disease characteristics and treatment compliance according to the age of pediatric FMF patients. This is a single-center, cross-sectional study. Between August and October 2016, patients who were diagnosed with FMF, participated to the study. 378 pediatric FMF patients were enrolled in the study. Among those, age at symptom onset was ≤ 5 years in 69%, 6–11 years in 26% and ≥ 12 years in 5%. Patients older than 12 years old at symptom onset, had significantly less frequent fever attacks than the patients from other age groups. Patients younger than 5 years old at symptom onset had significantly higher international severity scoring system for FMF (ISSF) than other patients. And M694V homozygosity was significantly more frequent in patients with younger age at symptom onset. Patients younger than 5 years old were using their drugs more regularly than the other age groups (p = 0.002). Drug compliance was 90.5% in patients ≤ 5 years, 64.4% in patients 6–11 years, 58.3% in patients ≥ 12 years. The disease characteristics of FMF may differ between patients with different age at symptom onset. Younger age at disease onset seems to be related with more severe course; thus these patients should be followed-up more closely. In addition, treatment compliance which is critical for prevention of amyloidosis in FMF should be questioned especially in adolescent patients.

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Funding

No funding support was received for this study.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, 06100, Ankara, Turkey

    Hafize Emine Sönmez, Selcan Demir, Erdal Sağ, Seza Özen & Yelda Bilginer

  2. Department of Pediatrics, Erzurum Training and Research Hospital, Erzurum, Turkey

    Pelin Esmeray

  3. Department of Pediatrics, Division of Rheumatology, Ankara Training and Research Hospital, University of Health Sciences, Ankara, Turkey

    Ezgi Deniz Batu

  4. Department of Pediatric Rheumatology, Şanlıurfa Training and Research Hospital, Şanlıurfa, Turkey

    Zehra Serap Arıcı

Authors
  1. Hafize Emine Sönmez
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  2. Pelin Esmeray
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  3. Ezgi Deniz Batu
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  4. Zehra Serap Arıcı
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  5. Selcan Demir
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  6. Erdal Sağ
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  7. Seza Özen
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  8. Yelda Bilginer
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Corresponding author

Correspondence to Yelda Bilginer.

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The authors report no conflicts of interest.

Ethical approval

The study was approved by the ethics committee of Hacettepe University (GO-16/187−48; approval date, 10th May 2016).

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Sönmez, H.E., Esmeray, P., Batu, E.D. et al. Is age associated with disease severity and compliance to treatment in children with familial Mediterranean fever?. Rheumatol Int 39, 83–87 (2019). https://doi.org/10.1007/s00296-018-4123-0

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  • DOI: https://doi.org/10.1007/s00296-018-4123-0

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