Summary

Familial Mediterranean fever (FMF) is characterised by recurrent, self-limited fever attacks and serositis. Severe liver involvement has rarely been reported. We present two FMF cases of a 55-year-old man and a 20-year-old woman in whom the prevailing manifestations were recurrent unexplained episodes of anicteric hepatitis (man) and recurrent severe jaundice (woman). A long-term history of recurrent self-limited episodes of fever was also claimed in both. After exclusion of infectious, malignant, autoimmune, and liver and biliary diseases, a diagnosis of FMF as confirmed by molecular analysis was established. The patients started colchicine 1 mg/day with immediate resolution of symptoms. During follow-up, no new episodes of fever and exacerbation of liver biochemical parameters have been recorded for 5 and 1 years. Physicians must keep FMF in mind in patients with recurrent episodes of unexplained severe liver impairment and fever and especially in regions like Mediterranean basin where hereditary periodic fever syndromes are common.