title: Anti-interleukin-1 treatment in 26 patients with refractory familial mediterranean fever.
abstract: OBJECTIVE
  To investigate the effect of anti-interleukin-1 (anti-IL-1) treatment on the frequency and severity of attacks and other disease-related clinical parameters and to evaluate the adverse effects associated with anti-IL-1 treatment in 26 patients with refractory familial mediterranean fever (FMF).
  
  
  METHODS
  The study included 26 FMF patients followed up in our centre using colchicine for 4 months to 30 years. The treatment was switched to anti-IL-1 treatment for various reasons; 20 cases were resistant to colchicine, 8 were intolerant to colchicine, and 3 had prolonged arthritis under colchicine. Clinical response was monitored through the number of attacks, and laboratory inflammation was monitored through erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A concentrations. Colchicine resistance was defined as at least two attacks/month together with C-reactive protein and serum amyloid A levels above the normal range between attacks. The colchicine dose was increased to 2 mg/day before they were considered colchicine-resistant.
  
  
  RESULTS
  24 patients used anakinra (100 mg/day), and 2 used canakinumab (150 mg/month), for -36 months. Sixteen patients with colchicine resistance had no attacks under anti-IL-1 treatment, and 4 had decreased frequency and duration of attacks. Seven of 8 patients intolerant to colchicine used anakinra, and 6 were attack-free under treatment, while 1 using canakinumab had attacks under treatment. One patient with prolonged arthritis used canakinumab but arthritis showed progression and the treatment was changed to IL-6 inhibitor. Three patients had injection site erythema and one had fatigue with anti-IL-1 treatment. Topical steroids with systemic antihistaminics were sufficient for symptom control in two cases, but canakinumab treatment was given due to severe injection site erythema in one case.
  
  
  CONCLUSIONS
  Anti-IL-1 agents are rational treatment modalities in patients resistant or intolerant to colchicine. Anti-IL-1 agents can control FMF attacks quite effectively and they have a promising role in the treatment of FMF.
authors.affiliation: a Division of Rheumatology , Yildirim Beyazit University , Ankara , Turkey.
authors.forename: Orhan
authors.initials: O
authors.lastname: Kucuksahin
authors.affiliation: b Department of Physical Medicine and Rehabilitation , Mustafa Kemal University Medical School , Hatay , Turkey.
authors.forename: Mustafa Turgut
authors.initials: MT
authors.lastname: Yildizgoren
authors.affiliation: c Department of Internal Medicine , Ankara University Medical School , Ankara , Turkey , and.
authors.forename: Ufuk
authors.initials: U
authors.lastname: Ilgen
authors.affiliation: d Department of Rheumatology , Ankara , Turkey.
authors.forename: Askin
authors.initials: A
authors.lastname: Ates
authors.affiliation: d Department of Rheumatology , Ankara , Turkey.
authors.forename: Gülay
authors.initials: G
authors.lastname: Kinikli
authors.affiliation: d Department of Rheumatology , Ankara , Turkey.
authors.forename: Murat
authors.initials: M
authors.lastname: Turgay
authors.affiliation: a Division of Rheumatology , Yildirim Beyazit University , Ankara , Turkey.
authors.forename: Sukran
authors.initials: S
authors.lastname: Erten
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000009:adverse effects / Q000276:immunology / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D003078:Colchicine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D010505:Familial Mediterranean Fever / Q000188:drug therapy*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D007375:Interleukin-1 / Q000037:antagonists & inhibitors / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D017472:Receptors, Interleukin-1 / Q000187:drug effects
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D017472:Receptors, Interleukin-1; C541220:canakinumab; D003078:Colchicine
keywords: Anakinra; Canakinumab; Colchicine; Familial meditarrenean fever; Interleukin-1