Background Idiopathic Recurrent Pericarditis can be challenging to treat in patients unresponsive to NSAIDs, aspirin, colchicine and immunosuppressive drugs. Patients become steroid dependent and tapering precipitates recurrences.
Objectives Report 2 adult cases of idiopathic recurrent pericarditis treated successfully with Anakinra.
Methods Chart review of 2 patients with idiopathic recurrent pericarditis treated with anakinra at the Keck Medical Center of USC. Literature review on treatment of idiopathic recurrent pericarditis with anakinra.
Results Case 1: 60-year-old Caucasian male had five episodes of idiopathic pericarditis in 2011. Serologic workup including ANA, anti-dsDNA, malignant and infectious workup was negative. Initially, patient responded to prednisone 0.4 mg/kg/day. Adding colchicine, azathioprine and methotrexate failed to prevent recurrence. Pericarditis developed whenever prednisone was tapered below 20 mg/day with bursts of CRP to 78 mg/dl. In 2012, Anakinra 100 mg sq daily resulted in immediate clinical response and normalization of CRP (1mg/dl). Prednisone and methotrexate were tapered with no recurrence. Gradually Anakinra was tapered to 3 times/week, then once a week, with no recurrence. Case 2: 37-year-old African American male had four episodes of recurrent pericarditis. He had positive ANA 1:320, but negative anti-dsDNA, anti-smith, negative infectious and malignancy workup. Initially, patient responded to prednisone 0.6 mg/kg/day and colchicine. Tapering steroids below 40 mg/day resulted in recurrent pericarditis. Sequential addition of hydroxychloroquine, methotrexate, mycophenolate mofetil, and azathioprine failed to prevent recurrence. Anakinra 100 mg sq daily resulted in prompt resolution of symptoms, normalization of acute phase reactants and allowed successful tapering of steroids. Anakinra is being slowly tapered over the past year, with no recurrence.
Conclusions Idiopathic recurrent pericarditis, which requires chronic corticosteroids, should be treated by adding another immunosuppressive agent. European Society of Cardiology guidelines recommend azathioprine, cyclophosphamide, methotrexate, hydroxychloroquine, cyclosporine or mycophenolate mofetil. Anakinra has demonstrated success in treating autoinflammatory and autoimmune diseases including FMF, TNF receptor associated periodic syndrome, rheumatoid arthritis and in patients with TRAPS mutation TNFRSF1A. This represented the possibility to decrease inflammation by blocking interleukin-1. Using this rationale we treated our patients. Picco et al reported three pediatric cases treated with anakinra, where its discontinuation resulted in recurrence. Vassilopoulos et al reported three adult cases, where two were not treated with immunosuppressive drugs before anakinra and one had recurrence after anakinra was tapered. The Double Blind Placebo Controlled Clinical Trial AIRTRIP, shows efficacy of anakinra in treating 11 patients with recurrent pericarditis over 14 months. It is unclear from this study if anakinra should be tapered or not. We suggest that once steroids and immunosuppressive drugs have been discontinued, anakinra should be gradually tapered over months, to avoid relapse. These experiences warrant further long term controlled trials in order to determine the efficacy and appropriate treatment regimen of anakinra for recurrent pericarditis.
Disclosure of Interest None declared