Abstract

Background Recurrent pericarditis (RP), however the etiology is unknown in the majority, may be observed in autoinflammatory diseases such as familial Mediterranean fever (FMF) and tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS). Colchicine has long been used to treat pericarditis related to FMF as well as patients with idiopathic recurrent pericarditis (IRP) (1). Alternative treatments have been reported for cases with colchicine resistant RP.Objectives: Objectives The aim is to present our data regarding anakinra treatment in recurrent pericarditis either related to FMF or idiopathic, who are resistant to colchicine. Methods Patients who had recieved anakinra with a diagnosis of recurrent pericarditis either idiopathic or secondary to FMF followed in our autoinflammatory disease center between 2014-2018 are evaluated retrospectively. From patients’ files, demographic and clinical features, response to other treatment approaches such as NSAID, corticosteroid, colchicine, were evaluated. All patients have been genetically screened for monogenic autoinflammatory diseases (MEFV, TRAPS, MVK, NLRP3, NOD2). Patients who had at least 3 attacks were administered anakinra 100 mg/day. Therapeutic efficacy, as well as side effect profile of anakinra is also assessed. Results There were 5 patients (3 male and 2 female) with the diagnosis of RP, 1 was related to FMF and 4 were idiopathic. The mean age of the group was 28±8 (range 20-40). All patients diagnosed with IRP were negative for autoinflammatory genetic screening, while a MEFV variant (K695R het.) was detected in the FMF patient. Median duration of follow-up was 30 months (range 11-129). In table 1, demographic and clinical features are given. The median number of recurrence was 6 before anakinra treatment. No episode of pericarditis was observed in any of the patients after the initiation of anakinra. The response to anakinra persisted even after the dose was reduced to 100 mg/alternate day in 3 patients, however in 2, recurrence of pericarditis was observed and anakinra was escalated to initial dose. It was possible to discontinue corticosteroid treatment in all patients. Currently all patients continue anakinra treatment. No side effect including injection site reaction, has been observed by now. Conclusion Anakinra seems to be a safe and effective treatment approach for colchicine resistant recurrent pericarditis. However recurrence may occur during dose tapering.

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