Recurrent Pericarditis: Is Immunotherapy the Answer?

INTERLEUKIN-1 ANTAGONISTS Interleukin-1 (IL-1) is a family of cytokines involved in both acute and chronic inflammation. IL-1β was found to be associated with autoimmune conditions, and is therefore used as a therapeutic target. IL-1 antagonists, such as anakinra, are used to treat autoimmune and autoinflammatory conditions such as familial Mediterranean fever [7]. Treatment with anakinra was previously tested in relatively small samples, but the results seemed promising. The treatment resulted in complete remission in corticosteroid-dependent patients and enabled discontinuation of corticosteroid treatment [8]. Another study reached similar conclusions, with complete and relatively fast remission (within a few days) [9]. An interventional double-blind placebo-controlled trial from Italy demonstrated significant reduction in pericarditis recurrence in colchicine-resistant and corticosteroiddependent patients [10]. Similar results were also seen in case reports, in both children [11] and adults [12]. A review conducted in 2016 by Lazaros et al. [13] reported that C-reactive protein (CRP) levels were normalized within an average of 7.1 days, and corticosteroids were discontinued within 62 days. It is important to note that discontinuation of anakinra leads to relapses in many cases, and therefore it seems that anakinra should be used as a long-term treatment [8,9]. The length of the treatment protocol is still unknown. The recommended dose is 1–2 mg/kg daily, up to 100 mg/daily in adults, for several months. It is not yet determined whether tapering down is needed [14]. Adverse effects described are injection P ericarditis is considered recurrent when reappearance of symptoms occurs after resolution of the initial acute attack, usually after 4 to 6 weeks. This complication is seen in up to 32% of acute pericarditis cases, and may increase up to 50% in untreated cases [1]. The pathogenesis and specific etiologies of recurrent pericarditis are still largely unknown, and therefore it is also referred to as idiopathic recurrent pericarditis. Autoimmunity, however, seems to play a crucial role. The autoimmune and autoinflammatory nature of the disease can be shown by the prevalence of autoantibodies in recurrent pericarditis patients [2] and in the effectiveness of immunosuppressant therapies in the disease [1]. The management of recurrent pericarditis is challenging. The efficacy of colchicine [3,4] and non-steroidal anti-inflammatory drugs (NSAID) [1] in prevention of recurrence is well-established, as are certain corticosteroid regimens in refractory cases [5]. In the case of corticosteroids, however, aside from their well-known adverse effects on other organ systems, they have also been associated with an increased recurrence of pericarditis, making them a double-edged sword [6]. Certain immunosuppressive therapies, such as azathioprine, have also been previously suggested [1]. In this article, we will discuss new emerging therapies for recurrent pericarditis. site reaction in up to 44% of patients [13]. Other rare adverse effects include transaminases elevation [9,10], herpes zoster, and optic neuropathy [10].