Successful management of colchicine resistant familial Mediterranean fever patients with a standardized canakinumab treatment protocol: a case series and literature review

  • Sanem Eren AkarcanEmail author
  • Seyda Dogantan
  • Neslihan Edeer Karaca
  • Guzide Aksu
  • Necil Kutukculer
Case Based Review


Familial Mediterranean fever (FMF) is the most common monogenic auto-inflammatory disease characterized by recurrent attacks of fever and serositis. Although colchicine is the first line treatment in FMF, 5–10% of patients do not respond to colchicine. Canakinumab, an anti-IL-1β monoclonal antibody, has been reported to be effective and safe in colchicine-resistant FMF patients, but the adequate duration and interval of treatment is still a matter of debate. Aim of this study was to evaluate the success of the standardized treatment protocol for canakinumab applied in our Pediatric Rheumatology Department in colchicine-resistant FMF cases with a review of the literature. Nine patients included in this study had indications for canakinumab use as colchicine resistance and recurrent corticosteroid need for pleural/pericardial effusions. Canakinumab was administered monthly for 6 months (initial treatment), bimonthly for 6 months (maintenance treatment), then treatment was discontinued. For the patients who developed a new attack after one-year treatment period, canakinumab was readministered with 3-month intervals (continuation treatment). The mean follow-up time beginning from the first canakinumab injection was 24.3 ± 10.2 (6–33) months. None of the patients had an attack during the first-year treatment. Four of the patients developed an attack 9.0 ± 2.9 (6–12) months after discontinuation of treatment and switched to the continuation treatment period, with no more attacks. We suggest that this standard protocol may be used successfully in colchicine-resistant FMF patients.


Familial Mediterranean fever Colchicine resistance Canakinumab Interleukin-1 receptor 


Author contributions

Study conception and design: SEA, GA, NK. Collection of data: contribution of all authors. Analysis and interpretation of data: SEA, NEK, NK. The literature data search and analysis: SEA, NK. Drafting of manuscript: SEA, SD, NK. Critical revision of manuscript: contribution of all authors. All authors approved the final version to be submitted for publication.

Compliance with ethical standards

Conflict of interest

Authors Eren Akarcan, Dogantan, Edeer Karaca, Aksu and Kutukculer declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (Ege University Medical Faculty Ethics Committee No: 19-4T/2) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Pediatric Immunology and Rheumatology DepartmentEge UniversityIzmirTurkey

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