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Improvement of Liver Involvement in Familial Mediterranean Fever After the Introduction of Canakinumab: A Case Report
Maria Grazia Massaro1, Maurizio Pompili1,2, Luca L Sicignano1
1Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
Abstract:
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.
Insights
Familial Mediterranean Fever (FMF) can cause liver issues. Interleukin-1 (IL-1) blockade with canakinumab, combined with colchicine, effectively improved liver enzymes and fibrosis in a patient with FMF.
Area of Science:
- Internal Medicine
- Genetics
- Hepatology
Background:
- Familial Mediterranean Fever (FMF) is an autoinflammatory disorder.
- Hepatic involvement in FMF ranges from elevated liver enzymes to cirrhosis, particularly in patients with the M694V MEFV mutation.
- Nonalcoholic steatohepatitis (NASH) can occur in FMF patients, even during colchicine treatment.
Observation:
- A 44-year-old Jewish woman with FMF developed NASH while on colchicine therapy.
- Diagnosis was confirmed by elastography and liver biopsy.
- Standard colchicine treatment (2.5 mg/day) was insufficient to manage the hepatic condition.
Findings:
- Combined therapy with canakinumab (an IL-1 blocker) and a reduced dose of colchicine (1.5 mg/day) was initiated.
- Within three months, transaminases normalized.
- Significant improvement in liver fibrosis was observed after six months of combined therapy.
Implications:
- Interleukin-1 (IL-1) blockade demonstrates potential in halting or mitigating liver involvement in FMF.
- This combined approach may offer a therapeutic option for severe hepatic disease in FMF patients unresponsive to colchicine alone.
- Further research is needed to establish the long-term efficacy and safety of IL-1 blockade in FMF-related liver disease.
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