Clinical Reviews in Allergy & Immunology

, Volume 45, Issue 1, pp 117–130

IL-1β Biological Treatment of Familial Mediterranean Fever

  • Alessandra Soriano
  • Elena Verecchia
  • Antonella Afeltra
  • Raffaele Landolfi
  • Raffaele Manna
Article

DOI: 10.1007/s12016-013-8358-y

Cite this article as:
Soriano, A., Verecchia, E., Afeltra, A. et al. Clinic Rev Allerg Immunol (2013) 45: 117. doi:10.1007/s12016-013-8358-y

Abstract

Familial Mediterranean fever (FMF) is a recessive, autosomal, auto-inflammatory disorder characterised by brief, recurring, self-limited episodes of fever and serositis resulting in abdominal, chest, joint and muscular pain; it is the most common of the periodic hereditary fevers and mostly affects Mediterranean populations. Daily administration of colchicine, a tricyclic alkaloid with anti-microtubule and anti-inflammatory properties, prevents the recurrence of FMF attacks and the development of secondary (AA) amyloidosis, the major long-tem complication of FMF. Colchicine is generally safe and well-tolerated; nevertheless, 5–10 % of FMF patients do not respond to conventional treatment, while another 2–5 % of patients are colchicine-intolerant because of toxicity issues, leading physicians to search for alternative therapeutic strategies. Recent new insights into the mechanisms of auto-inflammation add further proof to the efficacy of IL-1 targeting drugs in colchicine non-responder/intolerant FMF patients. A systematic study of relevant literature through PubMed/Medline was performed in order to identify publications reporting IL-1β biological treatment of FMF. Treatment methods, comorbidities, clinical response and side effects in literature case reports were analysed, as well as recent advances in the pathogenesis of auto-inflammation mechanisms in FMF and the causes of colchicine resistance or toxicity in common clinical practice. The paradigmatic experience of an FMF patient with severe FMF mutations (M694V/M694V) suffering from colchicine toxicity and successfully treated with anakinra is also reported. The present data show that anti-IL-1β biological treatment is actually a therapeutic option for FMF patients unresponsive or intolerant to colchicine or in FMF patients with concomitant vasculitis.

Keywords

Familial Mediterranean fever (FMF)Auto-inflammatory syndromesBiological agentsInterleukin (IL)-1βAnakinraCanakinumabRilonacept

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Alessandra Soriano
    • 1
    • 2
  • Elena Verecchia
    • 1
  • Antonella Afeltra
    • 2
  • Raffaele Landolfi
    • 3
  • Raffaele Manna
    • 1
    • 4
  1. 1.Periodic Fever Research Centre-National Reference Centre for FMFCatholic University of the Sacred HeartRomeItaly
  2. 2.Department of Clinical Medicine and RheumatologyCampus Bio-Medico UniversityRomeItaly
  3. 3.Department of Internal MedicineCatholic University of Sacred HeartRomeItaly
  4. 4.Department of Internal Medicine, Clinical Auto-Immunity Unit and Periodic Fever Research Centre-National Reference Centre for FMFCatholic University of Sacred HeartRomeItaly