title: Successful treatment with humanized anti-interleukin-6 receptor antibody (tocilizumab) in a case of AA amyloidosis complicated by familial Mediterranean fever.
abstract: Familial Mediterranean fever (FMF) is a well-known cause of secondary AA amyloidosis. Colchicine is generally considered to be the most effective treatment for FMF and FMF-associated amyloidosis, but the management of patients who are refractory to colchicine remains controversial. We encountered a 51-year-old Japanese man with suspected FMF, who had periodic fever with abdominal pain, polyarthritis, and nephropathy (serum creatinine of 1.9 mg/dL and 24-h protein excretion of 3.8 g). FMF was diagnosed by mutation analysis of the Mediterranean fever (MEFV) gene, which revealed that the patient was compound heterozygous for the marenostrin/pyrin variant E148Q/M694I. AA amyloidosis was diagnosed by renal and gastric biopsy. Colchicine was administered, but his arthritis persisted, and serum creatinine increased to 2.4 mg/dL. Therefore, a humanized anti-interleukin-6 receptor antibody (tocilizumab) was administered at a dose of 8 mg/kg on a monthly basis. Both arthritis and abdominal pain subsided rapidly, and C-reactive protein (CRP) decreased from 2.5 to 0.0 mg/dL. After 2 years, his serum creatinine was decreased to 1.5 mg/dL and proteinuria was improved to 0.3 g daily. In addition, repeat gastric biopsy showed a marked decrease of AA amyloidosis. This case suggests that tocilizumab could be a new therapeutic option for patients with FMF-associated AA amyloidosis if colchicine is not effective.
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hamanoue
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Koki
authors.initials: K
authors.lastname: Mise
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.|c Okinaka Memorial Institute for Medical Research , Tokyo , Japan.
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.affiliation: b Department of Pathology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.affiliation: b Department of Pathology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: d Departments of Medicine (Neurology and Rheumatology) and Pediatrics , Shinshu University School of Medicine , Matsumoto ; and the National Center of Neurology and Psychiatry , Tokyo , Japan.
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.affiliation: d Departments of Medicine (Neurology and Rheumatology) and Pediatrics , Shinshu University School of Medicine , Matsumoto ; and the National Center of Neurology and Psychiatry , Tokyo , Japan.
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ikeda
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.|c Okinaka Memorial Institute for Medical Research , Tokyo , Japan.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D002097:C-Reactive Protein / Q000235:genetics; D003078:Colchicine / Q000008:administration & dosage / Q000009:adverse effects; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007668:Kidney / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D000071198:Pyrin / Q000032:analysis / Q000235:genetics; D013270:Stomach / Q000473:pathology*; D016896:Treatment Outcome; D050257:Tubulin Modulators / Q000008:administration & dosage / Q000009:adverse effects
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007155:Immunologic Factors; C000604192:MEFV protein, human; D000071198:Pyrin; D050257:Tubulin Modulators; D002097:C-Reactive Protein; C502936:tocilizumab; D003078:Colchicine
keywords: AA amyloidosis; Familial Mediterranean fever; Humanized anti–interleukin-6 receptor antibody; Tocilizumab