Background: Approximately 5% of idiopathic recurrent pericarditis (IRP)patients are refractory or intolerant to NSAIDs, Colchicine and corticosteroids. The empiric treatment approach for these patients includes immunosuppression with Azathioprine (AZA)or immunomodulation with intravenous human immunoglobulin (IVIG). We assessed the efficacy and safety of long-term Anakinra treatment in refractory IRP patients after failure of prior immunosuppressive therapy and/or failure of IVIG. Methods: Clinical data of seven IRP patients were retrospectively analyzed. Treatment efficacy was determined by decrease of IRP recurrence and by the ability to withdraw or taper corticosteroids without a relapse. Safety was assessed by the occurrence of adverse events. Results: 7 IRP patients (4 male, median age 41)with a median disease duration of 4 years (range: 1.25–9 years)were treated with Anakinra (median treatment duration: 20 months). All patients were resistant or intolerant to NSAIDs, Prednisone, Colchicine and at least