Background/Purpose Idiopathic recurrent pericarditis (IRP) is a debilitating condition that can be recalcitrant to conventional therapy. Some patients develop steroid dependency with the attendant risks of systemic side effects and increased future recurrence. Anakinra is a recombinant human interleukin-1 receptor antagonist that reduces systemic inflammatory responses. The aim of this study was to evaluate the therapeutic role of anakinra in a series of adult patients with IRP refractory to conventional therapy.

Methods We retrospectively studied consenting patients with treatment refractory IRP who received anakinra, between January 2009 and November 2013. None of the patients had an identified systemic inflammatory rheumatic disease. The primary end points were symptom resolution and steroid discontinuation.

Results Nine patients were followed for a median of 16.8 (IQR 1.3-24) months. Study subjects were predominantly female (7 [78%]) with a median age of 53 (IQR 38 – 58) years. All 9 patients had failed maximum tolerated doses of NSAIDs, colchicine and prednisone (median dose 20 mg [IQR 15 – 22.5]). Additionally, 2 patients (22%) were on hydroxychloroquine, 2 (22%) on azathioprine and 1 (11%) on methotrexate. Primary symptom was chest pain in 9 (100%), with concurrent dyspnea in 5 (56%) patients. Symptom duration prior to anakinra initiation was 24 (IQR 13 – 71) months. Indication for anakinra was steroid sparing agent in 7 (78%) and symptom control in 2 (22%) patients. The dosage was 100 mg once daily via subcutaneous injection. Baseline left ventricular ejection fraction was 62% (IQR 59% – 67%).  Echocardiographic findings include pericardial effusion in 3 (33%), pericardial thickening in 6 (67%), and constrictive physiology in 4 (44%). Pericardial enhancement on cardiac MRI was seen in 7 (78%) patients. All 9 patients (100%) had prompt symptom improvement with complete resolution in 8 (89%) and partial resolution in 1 (11%) patient. At last follow up, ­­­­all (100%) patients had discontinued NSAIDs and colchicine, 5 (56%) patients discontinued and 4 (44%) had reduced prednisone dosage. 4 out of 5 patients (80%) discontinued concomitant immunsuppressive agents. 1 patient remained on low dose hydroxychloroquine. All patients remained on anakinra at the end of follow up. The only reported side effect was transient injection site reaction in 4 (44%) patients. In 2 (22%) patients, attempted anakinra weaning was unsuccessful due to symptoms flare after 6 weeks. 

Conclusion Anakinra is an effective alternative agent for the management of steroid dependent IRP. It can provide remarkable symptomatic amelioration, avoid steroid dependency, and is associated with minimal side effects.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/anakinra-a-promising-new-therapy-for-idiopathic-recurrent-pericarditis/