title: Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis.
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.
authors.affiliation: Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, 06100, Ankara, Turkey.
authors.forename: Z Birsin
authors.initials: ZB
authors.lastname: Özçakar
authors.affiliation: Department of Pediatrics, Division of Pediatric Rheumatology, Ankara University School of Medicine, Ankara, Turkey.
authors.forename: Semanur
authors.initials: S
authors.lastname: Özdel
authors.affiliation: Department of Pediatrics, Division of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey.
authors.forename: Songül
authors.initials: S
authors.lastname: Yılmaz
authors.affiliation: Department of Pediatrics, Division of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey.
authors.forename: E Didem
authors.initials: ED
authors.lastname: Kurt-Şükür
authors.affiliation: Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, 06100, Ankara, Turkey.
authors.forename: Mesiha
authors.initials: M
authors.lastname: Ekim
authors.affiliation: Department of Pediatrics, Division of Pediatric Rheumatology & Nephrology, Ankara University School of Medicine, Çınar Sitesi 5.Blok No:62, 06530, Ümitköy, Turkey. fyalcin@medicine.ankara.edu.tr.
authors.forename: Fatoş
authors.initials: F
authors.lastname: Yalçınkaya
mesh_terms: D000293:Adolescent; D000686:Amyloidosis / Q000188:drug therapy* / Q000209:etiology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D002648:Child; D003078:Colchicine / Q000627:therapeutic use*; D004351:Drug Resistance; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D053590:Interleukin 1 Receptor Antagonist Protein; C541220:canakinumab; D003078:Colchicine
keywords: Amyloidosis; Anakinra; Canakinumab; Familial Mediterranean fever; Treatment