TY - JOUR
T1 - Improvement of liver involvement in familial Mediterranean fever after introduction of canakinumab: a case report
AU - Massaro, Maria Grazia
AU - Pompili, Maurizio
AU - Sicignano, Ludovico Luca
AU - Pizzolante, Fabrizio
AU - Vecchio, Fabio Maria
AU - Rigante, Donato
AU - Manna, Raffaele
PY - 2020
Y1 - 2020
N2 - 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.
AB - 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.
KW - Canakinumab
KW - Familial Mediterranean fever
KW - Canakinumab
KW - Familial Mediterranean fever
UR - http://hdl.handle.net/10807/160787
U2 - 10.4084/MJHID.2020.059
DO - 10.4084/MJHID.2020.059
M3 - Article
SN - 2035-3006
VL - 2020
SP - 1
EP - 5
JO - Mediterranean Journal of Hematology and Infectious Diseases
JF - Mediterranean Journal of Hematology and Infectious Diseases
ER -