Case report A 15-year-old girl with familial Mediterranean fever (FMF) was admitted to our hospital because of the persistence of recurrent fever attacks (twice a month), accompanied by chest and abdominal pain with headache, despite taking colchicine at a high dose (2 mg/kg per day). She suffered severe discomfort and complained of several school absences. Acute phase reactants [erythrocyte sedimentation rate (ESR) 110, C-reactive protein (CRP) 43 mg%] and serum interferon-γ (125.4 pg/ml) were high during the crises. There was no proteinuria, but given the severity of the clinical picture, we performed a rectal biopsy to definitely exclude the presence of amyloidosis and it was negative. Anakinra was started at 50 mg/day (1 mg/kg per day) subcutaneously, without stopping colchicine. Approval was obtained from an Ethics Committee (Burlo Garofolo Health Authority). In the following 3 months the patient developed only three mild episodes of abdominal pain without fever, which resolved in a few hours. During these episodes acute phase reactants were negative. Moreover, serum interferon-γ was low (23 pg/ml). After 3 months, anakinra was discontinued to confirm its real efficacy, without stopping colchicine. Following this interruption, the girl complained of eight fever attacks in 3 months accompanied by abdominal pain, vomiting, chest pain, and headache. Anakinra was reintroduced. Since then (15 months of follow-up), the patient has been in good health, without any fever attacks or abdominal or chest pain. There have been no adverse effects due to anakinra, apart from minor local stinging and erythema at the injection sites, which gradually diminished. Discussion 3 5 3 3 3 6 2 4 1 5 1 3 5