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The association between familial Mediterranean fever and incident cirrhosis: A population-based matched cohort study
Michal Carmiel-Haggai1, Rula Daood2, Fadi Fassan2
1Liver Unit, Galilee Medical Centre, Nahariya, Israel; The Azrieli's Faculty of Medicine, Bar-Ilan University, Safed, Israel.
Objective:
The association between FMF and incident liver cirrhosis is not widely studied. In this study, we aimed to examine the association between FMF and incident liver cirrhosis in a population-based cohort.
Methods:
Patients with FMF aged≥18 in the Maccabi Healthcare Services (MHS) database were identified according to ICD-9 code 277.31 between January 1st, 2000 and December 31st, 2022. A control group was 1:1 age and gender-matched. Patients with chronic liver disease or cirrhosis were excluded, as well as patients who were treated with methotrexate, amiodarone or tamoxifen and patients with less than 12 months of follow-up. Incident cirrhosis was defined as a new diagnosis of cirrhosis according to ICD-9 code (571.5), newly diagnosed major cirrhotic complications or liver transplantation. The Cox proportional hazards models were used to examine the association between FMF and incident cirrhosis and the Kaplan-Meier curves to study the event-free survival.
Results:
Incident cirrhosis was detected among 2.1% and 0.4% in the study and control groups, respectively, P<0.01. Being in the FMF group was associated with a significantly increased risk of incident cirrhosis (HR=2.60, 95% CI: 1.54-4.38, P<0.01). At 7 years, the cirrhosis-free survival rate was 98.2% in the study group and 99.6% in the control group (P<0.01).
Conclusion:
FMF was associated with incident cirrhosis, irrespective of the traditional risk factors for metabolic syndrome, suggesting the contribution of the inflammatory state to the development of cirrhosis.
Insights
Individuals with Familial Mediterranean Fever (FMF) have a significantly higher risk of developing liver cirrhosis. This inflammatory condition may contribute to cirrhosis development, independent of metabolic syndrome factors.
Area of Science:
- Hepatology
- Rheumatology
- Epidemiology
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- The link between FMF and liver cirrhosis is not well-established.
- Inflammation is a known factor in liver disease progression.
Purpose of the Study:
- To investigate the association between FMF and the incidence of liver cirrhosis.
- To analyze cirrhosis risk in a large, population-based cohort with FMF.
Main Methods:
- Retrospective cohort study using Maccabi Healthcare Services data (2000-2022).
- Identified FMF patients (ICD-9: 277.31) and matched controls.
- Excluded patients with pre-existing liver disease or specific medications; required ≥12 months follow-up.
- Defined incident cirrhosis by diagnosis code (571.5), complications, or liver transplant.
- Utilized Cox proportional hazards models and Kaplan-Meier survival analysis.
Main Results:
- Incident cirrhosis occurred in 2.1% of FMF patients vs. 0.4% of controls (P<0.01).
- FMF diagnosis was associated with a 2.60-fold increased risk of incident cirrhosis (HR=2.60, 95% CI: 1.54-4.38, P<0.01).
- Cirrhosis-free survival at 7 years was lower in the FMF group (98.2%) compared to controls (99.6%, P<0.01).
Conclusions:
- FMF is significantly associated with an increased risk of developing incident liver cirrhosis.
- This association persists independently of metabolic syndrome risk factors.
- The chronic inflammatory state in FMF may play a crucial role in cirrhosis development.
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