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Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis

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Abstract

Colchicine is the standard treatment in familial Mediterranean fever (FMF) patients. New treatment strategies are needed in FMF patients who were unresponsive to colchicine therapy or who had developed amyloidosis. The aim of this study was to present clinical-laboratory features and treatment responses of pediatric FMF patients that were treated with anti-IL-1 therapies. Files of patients who had been followed in our department with diagnosis of FMF were retrospectively evaluated. Patients that have been receiving anti-IL-1 therapies (anakinra or canakinumab) were included to the study. All patients were interpreted with respect to the demographic data, clinical and laboratory features of the disease, genetic analysis of MEFV mutations and treatment responses. Among 330 currently registered FMF patients, 13 patients were included to the study. Seven of them received anti-IL-1 therapy due to colchicine resistance and 6 due to FMF-related amyloidosis (1 of them with nephrotic syndrome, 2 with chronic kidney disease, 3 with renal transplantation). In all treated patients, attacks completely disappeared or decreased in frequency; partial remission occured in nephrotic syndrome patient; and their life quality improved. Anti-IL-1 therapies can be successfully used in colchicine-resistant FMF patients and patients with amyloidosis during childhood and adolescent period without major side effects.

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Conflict of interest

All authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, 06100, Ankara, Turkey

    Z. Birsin Özçakar & Mesiha Ekim

  2. Department of Pediatrics, Division of Pediatric Rheumatology, Ankara University School of Medicine, Ankara, Turkey

    Semanur Özdel

  3. Department of Pediatrics, Division of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey

    Songül Yılmaz & E. Didem Kurt-Şükür

  4. Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, Çınar Sitesi 5.Blok No:62, 06530, Ümitköy, Turkey

    Fatoş Yalçınkaya

Authors
  1. Z. Birsin Özçakar
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  2. Semanur Özdel
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  3. Songül Yılmaz
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  4. E. Didem Kurt-Şükür
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  5. Mesiha Ekim
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  6. Fatoş Yalçınkaya
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Correspondence to Fatoş Yalçınkaya.

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Özçakar, Z.B., Özdel, S., Yılmaz, S. et al. Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis. Clin Rheumatol 35, 441–446 (2016). https://doi.org/10.1007/s10067-014-2772-2

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  • DOI: https://doi.org/10.1007/s10067-014-2772-2

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