Successful treatment of adult patients with idiopathic recurrent pericarditis with an interleukin-1 receptor antagonist (anakinra)

Abstract

Idiopathic recurrent pericarditis (IRP) is observed in 10–30% of cases after a first episode of acute pericarditis, whereas a second recurrence appears in anevenhigher rate (~50%) [1,2]. Although severalmechanisms have been suggested to explain recurrence, most of them appear simply contributory. Today, there is increasing evidence that autoimmune and autoinflammatory pathways aremainly involved in its pathogenesis [3,4]. The optimal regimen for treating IRP has not been established [1]. Several medications either alone or in combination have been tested including nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin, colchicine, corticosteroids, and immunosuppressive agents. To date, the only available medication proved in randomized trials to decrease the recurrence rate is colchicine [2]. However, even with colchicine, a substantial proportion of patients experience recurrences [2]. Recently, Picco and et al. reported the successful use of an interleukin1β receptor antagonist anakinra in three children with resistant IRP [4]. Here, we present our experience in three adults with drug resistant or intolerant IRP. All patients were informed in detail regarding the management with anakinra and gave informed consent. Case#1: A 26-year-oldmale had suffered 8 episodes of IRP since 2004. He has received various regimens including NSAIDs, colchicine, steroids and azathioprine (Fig. 2A). In October 2006 treatment with prednisone was instituted but his symptoms recurred every time the dose was tapered below 15mg/day. Unfortunately, the patient developed steroidrelated complications including glaucoma, osteopenia and proximal myopathy of the lower extremities. In his latest admission inMarch 2010, the patient was started on anakinra (150 mg/day subcutaneously, SC) as monotherapy for 3 months and steroidswere tapered off. Administration of anakinrawas followed byan immediate and dramatic clinical response International Journal of Cardiology 160 (2012) 66–77