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Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report.
Massaro, Maria Grazia; Pompili, Maurizio; Sicignano, Luca L; Pizzolante, Fabrizio; Verrecchia, Elena; Vecchio, Fabio M; Rigante, Donato; Manna, Raffaele.
Afiliação
  • Massaro MG; Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Pompili M; Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Sicignano LL; Università Cattolica del Sacro Cuore, Rome, Italy.
  • Pizzolante F; Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Verrecchia E; Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Vecchio FM; Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Rigante D; Department of Pathology, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
  • Manna R; Università Cattolica del Sacro Cuore, Rome, Italy.
Mediterr J Hematol Infect Dis ; 12(1): e2020059, 2020.
Article em En | MEDLINE | ID: mdl-32952970
Hepatic involvement in familial Mediterranean fever (FMF) ranges from a nonspecific increase in liver enzymes to cryptogenic cirrhosis, and the liver is mostly involved in patients bearing the M694V MEFV mutation in homozygosis. A 44-year-old Jewish woman with FMF developed nonalcoholic steatohepatitis during colchicine treatment (2,5 mg per day), confirmed by both elastography and liver biopsy. Therefore, combined therapy with the interleukin-1 (IL-1) blocking agent canakinumab (150 mg every four weeks) and colchicine (at a reduced dose of 1.5 mg per day) was started. Three months later, transaminases became normal, and after further six months, there was a marked improvement of liver fibrosis. IL-1 blockade has the power to halt or mitigate liver involvement in FMF patients. However, further experience is required to assess its therapeutic potential in the most severe patients with the hepatic disease who are partially responsive to long-term prophylaxis with colchicine.
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Texto completo: 1 Base de dados: MEDLINE Idioma: En Revista: Mediterr J Hematol Infect Dis Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Itália

Texto completo: 1 Base de dados: MEDLINE Idioma: En Revista: Mediterr J Hematol Infect Dis Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Itália