title: Biologic therapy in familial Mediterranean fever.
abstract: Familial Mediterranean fever (FMF) is the most common autoinflammatory hereditary disease characterized by self-limited attacks of fever and serositis. Although colchicine is the gold standard treatment for the attacks ∼10% of cases of FMF are resistant or intolerant to effective doses of colchicine. In such cases, however, there are increasing numbers of case reports or clinical trials treated by biologic agents which directly target the proinflammatory cytokines. Anti-interleukin-1 (IL-1) treatment has proven beneficial in improving the inflammation in terms of clinical manifestations and laboratory findings in clinical trials. Furthermore, anti-tumor necrosis factor treatment has also revealed the efficacy and safety in patients with colchicine-resistant FMF. More recently, cases of successful treatment with IL-6 inhibitor, tocilizumab (TCZ), has been reported from Japan and Turkey. Of note, TCZ may be preferable in the treatment as well as the prevention of secondary amyloidosis of FMF patients since it significantly suppresses acute inflammatory response. In the present review, we summarize the literatures regarding the efficacy of biologic therapy in colchicine-resistant or -intolerant patients with FMF.
authors.affiliation: a Unit of Translational Medicine, Department of Immunology and Rheumatology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan and.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Koga
authors.affiliation: b Department of Rheumatology and Clinical Research Center , Nagasaki Medical Center , Omura , Japan , Nagasaki.
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Migita
authors.affiliation: a Unit of Translational Medicine, Department of Immunology and Rheumatology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan and.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kawakami
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use*; D003078:Colchicine / Q000627:therapeutic use*; D010505:Familial Mediterranean Fever / Q000188:drug therapy*; D006801:Humans; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D001688:Biological Products; C502936:tocilizumab; D003078:Colchicine
keywords: Auto-inflammation; Familial Mediterranean fever; IL-1β inhibitor; IL-6 inhibitor; TNF inhibitor