Improvement of liver involvement in familial Mediterranean fever after introduction of canakinumab: a case report

• Main Authors: Maria Grazia Massaro, Maurizio Pompili, Ludovico Luca Sicignano, Fabrizio Pizzolante, Elena Verrecchia, Fabio Maria Vecchio, Donato Rigante, Raffaele Manna
• Format: Article
• Language: English
• Published: PAGEPress Publications 2020-08-01
• Series: Mediterranean Journal of Hematology and Infectious Diseases
• Subjects: Familial Mediterranean fever, autoinflammation, periodic fever, steatosis, hepatitis, colchicine, interleukin-1, innovative biotechnologies, anakinra, canakinumab, personalized medicine
• Online Access: https://www.mjhid.org/index.php/mjhid/article/view/4272

A 44-year-old Jewish woman with familial Mediterranean fever (FMF) developed non-alcoholic steato-hepatitis during colchicine treatment (2,5 mg per day), confirmed by both elastographic study and liver biopsy. A combined therapy with the interleukin-1 (IL-1) blocking agent canakinumab (150 mg every 4 weeks) and colchicine (at a reduced dose of 1.5 mg per day) was started. Three months later transaminases became normal, and six months later there was a marked improvement of liver fibrosis on the elastographic study. Hepatic involvement in FMF occurs ranging from nonspecific increase in liver enzymes to cryptogenic cirrhosis. Liver is mostly involved in patients bearing the homozygous M694V MEFV mutation, as in our case. IL-1 blockade has the power to halt or mitigate liver involvement in FMF patients, though further experience is required to assess its therapeutic potential in the most severe patients with hepatic disease who are partially responsive to long-term colchicine.