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Factors Associated With Successful Withdrawal of Biologic Agents in Children With Colchicine-Resistant Familial

Özen Taş1, Fatma Aydın1, Müge Sezer2

  • 1From the Division of Pediatric Rheumatology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.

Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases
|September 3, 2024
PubMed
Abstract

Insights

For colchicine-resistant Familial Mediterranean Fever (FMF), lower disease severity scores and absence of exertional leg pain, subclinical inflammation, and comorbidities predict successful discontinuation of anti-interleukin-1 (anti-IL-1) biological agents. These factors are key for managing FMF patients. Keywords: Familial Mediterranean Fever, FMF, colchicine-resistant, anti-IL-1, biological therapy, disease severity.

Area of Science:

  • Rheumatology
  • Immunology
  • Genetics

Background:

  • Familial Mediterranean Fever (FMF) is a monogenic autoinflammatory disease primarily treated with colchicine.
  • A subset of patients (5%-10%) exhibit inadequate response to colchicine, necessitating alternative therapies.
  • Anti-interleukin-1 (anti-IL-1) agents represent crucial therapeutic options for colchicine-resistant FMF.

Purpose of the Study:

  • To identify factors associated with the discontinuation of anti-IL-1 agents in FMF patients.
  • To characterize colchicine-resistant FMF patients requiring biological therapy.
  • To investigate predictors for successful withdrawal of biological agents in pediatric FMF.

Main Methods:

  • A comparative study of demographic, clinical characteristics, and disease severity (using the International Severity Scoring System for FMF - ISSF) was conducted.
  • Data from 64 colchicine-resistant FMF patients treated between 2012 and 2022 at two referral centers were analyzed.
  • Patients receiving anti-IL-1 treatment (anakinra or canakinumab) were categorized into those whose treatment was continued versus discontinued.

Main Results:

  • The median duration of biological treatment was 39 months.
  • Anti-IL-1 treatment was discontinued in 35.9% of patients.
  • Higher ISSF scores, exertional leg pain, subclinical inflammation, and comorbidities were significantly associated with inability to discontinue biological therapy (p < 0.05).

Conclusions:

  • Low ISSF scores prior to biological treatment are associated with successful discontinuation.
  • Absence of exertional leg pain, subclinical inflammation, and comorbidities are favorable factors for discontinuing anti-IL-1 agents.
  • These findings aid in optimizing biological agent use in pediatric colchicine-resistant FMF.

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