Objectives: Familial Mediterranean fever (FMF) is characterised by recurrent attacks of fever and serositis. It may affect the peritoneum, pleura, synovia and the skin. Usually the liver is intact in FMF. Recently, this concept was challenged by some groups which claimed that hepatitis is a feature of FMF and that non-alcoholic liver disease (NAFLD) and cryptogenic cirrhosis are more common among FMF patients. Scope of this paper is to critically review the relevant literature and to answer the question whether or not the liver is involved in FMF.
Methods: We used Medline, Embase, Scopus and Web of Science database for searching articles dealing with FMF and the liver since 1960. We also reviewed some manuscripts which were not identified by the above searching engines.
Results: Some cases reported that hepatitis is a feature of FMF based upon transaminase elevations without liver biopsy. Due to this questionable diagnosis and the paucity of similar reports, it seems that hepatitis is not a feature of FMF. Cryptogenic cirrhosis is considered as the end stage of NAFLD. Since NAFLD is prevalent in 25% of the general population it is more plausible to relate the occurrence of cryptogenic cirrhosis in FMF patients to NAFLD rather than to FMF. M694V mutation carriage was relatively more frequent among FMF patients with cryptogenic cirrhosis or "hepatitis".
Conclusions: The literature review indicates that FMF and liver disease are not generally associated. However, carriage of M694V mutations may play a role in the pathogenesis of liver disease.