Cargando…

Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report

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 steatohepati...

Descripción completa

Detalles Bibliográficos
Autores principales: Massaro, Maria Grazia, Pompili, Maurizio, Sicignano, Luca L., Pizzolante, Fabrizio, Verrecchia, Elena, Vecchio, Fabio M., Rigante, Donato, Manna, Raffaele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7485480/
https://www.ncbi.nlm.nih.gov/pubmed/32952970
http://dx.doi.org/10.4084/MJHID.2020.059
_version_ 1783581157977227264
author Massaro, Maria Grazia
Pompili, Maurizio
Sicignano, Luca L.
Pizzolante, Fabrizio
Verrecchia, Elena
Vecchio, Fabio M.
Rigante, Donato
Manna, Raffaele
author_facet Massaro, Maria Grazia
Pompili, Maurizio
Sicignano, Luca L.
Pizzolante, Fabrizio
Verrecchia, Elena
Vecchio, Fabio M.
Rigante, Donato
Manna, Raffaele
author_sort Massaro, Maria Grazia
collection PubMed
description 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.
format Online
Article
Text
id pubmed-7485480
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher Università Cattolica del Sacro Cuore
record_format MEDLINE/PubMed
spelling pubmed-74854802020-09-17 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 Mediterr J Hematol Infect Dis Case Report 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. Università Cattolica del Sacro Cuore 2020-09-01 /pmc/articles/PMC7485480/ /pubmed/32952970 http://dx.doi.org/10.4084/MJHID.2020.059 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Massaro, Maria Grazia
Pompili, Maurizio
Sicignano, Luca L.
Pizzolante, Fabrizio
Verrecchia, Elena
Vecchio, Fabio M.
Rigante, Donato
Manna, Raffaele
Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title_full Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title_fullStr Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title_full_unstemmed Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title_short Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
title_sort improvement of liver involvement in familial mediterranean fever after the introduction of canakinumab: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7485480/
https://www.ncbi.nlm.nih.gov/pubmed/32952970
http://dx.doi.org/10.4084/MJHID.2020.059
work_keys_str_mv AT massaromariagrazia improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT pompilimaurizio improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT sicignanolucal improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT pizzolantefabrizio improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT verrecchiaelena improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT vecchiofabiom improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT rigantedonato improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport
AT mannaraffaele improvementofliverinvolvementinfamilialmediterraneanfeveraftertheintroductionofcanakinumabacasereport