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IL-1β Biological Treatment of Familial Mediterranean Fever

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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.

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Acknowledgements

The authors thank Dr. Craig Peritz for language revision.

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Authors and Affiliations

  1. Periodic Fever Research Centre-National Reference Centre for FMF, Catholic University of the Sacred Heart, Rome, Italy

    Alessandra Soriano, Elena Verecchia & Raffaele Manna

  2. Department of Clinical Medicine and Rheumatology, Campus Bio-Medico University, Rome, Italy

    Alessandra Soriano & Antonella Afeltra

  3. Department of Internal Medicine, Catholic University of Sacred Heart, L.go F. Vito, 1, 00168, Rome, Italy

    Raffaele Landolfi

  4. Department of Internal Medicine, Clinical Auto-Immunity Unit and Periodic Fever Research Centre-National Reference Centre for FMF, Catholic University of Sacred Heart, L.go F. Vito, 1, 00168, Rome, Italy

    Raffaele Manna

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  1. Alessandra Soriano
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  2. Elena Verecchia
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Correspondence to Raffaele Manna.

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Soriano, A., Verecchia, E., Afeltra, A. et al. IL-1β Biological Treatment of Familial Mediterranean Fever. Clinic Rev Allerg Immunol 45, 117–130 (2013). https://doi.org/10.1007/s12016-013-8358-y

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