Familial mediterranean fever medical therapy

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Familial Mediterranean Fever Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Clinical practice guidelines direct treatment[1]:

03. "Treatment with colchicine should start as soon as a clinical diagnosis is made"

17. "In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and IL-1-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain"

IL-1-blockade

A systematic review of IL-1 blockade with anakinra, canakinumab, and rilonacept found only one randomized controlled trial.[2]

  • anakinra: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 3.5 with placebo to 1.7 with anakinra.[3] Average age was 37 years and 83% of subjects were homozygous for M694V. This trial was published and registered (NCT01705756).
  • canakinumab: no randomized controlled trials.
  • rilonacept: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 2 with placebo to 0.77 with rilonacept.[4] Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).

References

  1. Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G; et al. (2016). "EULAR recommendations for the management of familial Mediterranean fever". Ann Rheum Dis. 75 (4): 644–51. doi:10.1136/annrheumdis-2015-208690. PMID 26802180.
  2. van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S (2016). "Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature". Biologics. 10: 75–80. doi:10.2147/BTT.S102954. PMC 4831592. PMID 27110096.
  3. Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity S; et al. (2017). "Anakinra for Colchicine-Resistant Familial Mediterranean Fever: A Randomized, Double-Blind, Placebo-Controlled Trial". Arthritis Rheumatol. 69 (4): 854–862. doi:10.1002/art.39995. PMID 27860460.
  4. Hashkes PJ, Spalding SJ, Giannini EH, Huang B, Johnson A, Park G; et al. (2012). "Rilonacept for colchicine-resistant or -intolerant familial Mediterranean fever: a randomized trial". Ann Intern Med. 157 (8): 533–41. doi:10.7326/0003-4819-157-8-201210160-00003. PMID 23070486.