The association of Familial Mediterranean Fever and cryptogenic cirrhosis

Baskın, Esra; Özçay, Figen; Bayrakci, US; Hoşnut, Ferda Özbay; Gülleroğlu, Kaan

doi:10.1186/1546-0096-6-s1-p205

Cited by 2 publications

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“…Baskin et al 28 further described a 9‐year‐old boy carrying homozygous M694V mutations, who suffered from a long delay between onset of symptoms and FMF diagnosis with subsequent initiation of colchicine therapy. Hepatosplenomegaly led to the discovery of cirrhosis upon liver biopsy, with no obvious aetiology other than FMF.…”

Section: Resultsmentioning

confidence: 99%

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Fraisse

Savey

Hentgen

et al. 2020

Liver International

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Introduction: Familial Mediterranean fever (FMF), the most frequent autoinflammatory disease, is caused by mutations in the MEFV gene. It is characterized by recurrent febrile attacks of polyserositis. Liver abnormalities may develop during its course, but they remain poorly defined.Objective: To describe liver involvement in FMF patients. Methods:A systematic search was conducted through PubMed/Medline and Embase from 1946 to January 2020. All articles describing children and adults with FMF and liver involvement were included. Patients with amyloidosis were excluded. The selected full-text articles were independently reviewed by three investigators.Results: Forty-three articles were identified, of which 20 articles with a total of 99 patients were included: 74 adults, 23 children and two patients of unknown age. Ten patients had cryptogenic cirrhosis, 48 had nonalcoholic fatty liver disease (NAFLD), four had Budd-Chiari syndrome (BCS), 12 had isolated hyperbilirubinaemia and 25 had elevated liver enzymes. Conclusion:Despite a low prevalence of metabolic risk factors, FMF may be associated with NAFLD and cryptogenic cirrhosis as a consequence of chronic or recurrent inflammation. FMF patients should be regularly screened for liver injury. The latter may be prevented and treated by daily colchicine intake. The evidence was insufficient to establish an association with BCS, hyperbilirubinaemia or autoimmune hepatitis. K E Y W O R D Scolchicine, cytolysis, familial Mediterranean fever, inflammation, nonalcoholic fatty liver disease, nonalcoholic steatohepatitis

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Section: Resultsmentioning

confidence: 99%

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Fraisse

Savey

Hentgen

et al. 2020

Liver International

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Prevalence of Familial Mediterranean Fever in Children with Cryptogenic Cirrhosis

Varol

Tabel

Yoloğlu

et al. 2021

Journal of Tropical Pediatrics

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Background Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by abdominal and chest pain and recurrent fever due to inflammation in the serosal membranes such as peritoneum, pleura and synovia. In FMF, recurrent inflammatory cytokine production may lead to cirrhosis. The aim of this study was to determine the prevalence of FMF in children with cryptogenic cirrhosis and it was found to be high, to add FMF among the etiological causes of cirrhosis. Materials and methods This prospective cohort study conducted at the Hospital of İnönü University, Malatya, Turkey. In this study, 44 patients diagnosed with cryptogenic cirrhosis by biopsy, in the Pediatric Gastroenterology, Hepatology and Nutrition Clinic, were included, after the other reasons that may cause chronic liver disease were excluded. MEVF gene analysis was performed for all patients with cryptogenic cirrhosis. Results FMF genetic mutation was detected in 9 (20%) of 44 patients. M694V mutation was detected in one patient (2.27%) and E148Q homozygous mutation was detected in one patient (2.27%). Various other heterozygous mutations were detected in seven other patients. Homozygous and heterozygous R202Q mutations were detected in one patient. Conclusion We suggest that FMF plays a role in the etiologic differential diagnosis of cryptogenic cirrhosis.

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The association of Familial Mediterranean Fever and cryptogenic cirrhosis

Cited by 2 publications

References 0 publications

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Non‐amyloid liver involvement in familial Mediterranean fever: A systematic literature review

Prevalence of Familial Mediterranean Fever in Children with Cryptogenic Cirrhosis

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