The tale of refractory recurrent pericarditis

Abstract

Among pericardial syndromes, acute pericarditis is the most common with an estimated incidence of ~ 28 cases per 100,000 subjects per year in the Western world where data are available [1]. Apart from life-threatening cases namely those complicated with cardiac tamponade (1.2% in idiopathic forms), acute idiopathic pericarditis is a benign condition in terms of mortality [2]. In contrast, this does not hold true in terms of morbidity since a proportion of cases has a problematic course due to recurrent attacks in the long run [1, 3]. Actually, in the specific context of recurrent pericarditis (RP) a particularly troublesome entity consists of the so-called glucocorticoid-dependent colchicine-resistant pericarditis accounting for 5–10% of the overall group of patients with RP [4]. The latter unfortunate patients continue to depict recurrences despite colchicine administration and are not able to taper or withdraw glucocorticoids without experiencing a further recurrence. Moreover, another highly concerning subset of patients include those with incessant pericarditis who over and above the difficulties in treatment exhibit a high rate of progression to constrictive pericarditis [4]. The management of patients with pericardial syndromes in general and RP, in particular, has been rather empirical until the beginning of the twenty-first century and has been summarized in the 2004 European Society of Cardiology (ESC) first Guidelines focusing on pericardial diseases [5]. It is quite impressive that for a definitely not uncommon disorder as acute pericarditis the available medications at that time consisted exclusively of non-steroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids whereas the use of colchicine was at a very early stage. Immunosuppression despite the complete lack of convincing evidence was reserved for difficult cases whereas pericardiectomy was the last resort for refractory cases. In 2015 the release of the second ESC guidelines highlighted the outstanding progress accomplished in recent years on pericardial syndromes [1]. Specifically, for RP the pathophysiology has been better characterized with the inclusion of autoinflammation among the underlying mechanisms further to infection and autoimmunity. Another novelty in the most recent guidelines was the detailed information on the contribution of multimodality imaging in the diagnostic evaluation and treatment of RP with emphasis on the role of second-level imaging such as computed tomography and cardiac magnetic resonance imaging (cMRI). Last but certainly not least the most recent guidelines discuss the new therapeutic strategies for refractory RP. In specific, the role of colchicine has been definitely established based on the results of multicentre randomized clinical trials. Moreover, the recommended dose of prednisone is now 0.2–0.5 mg/kg/day (or equivalent) which is much lower than previously described. Finally, new therapeutic choices are discussed such as the interleukin-1 (IL-1) antagonist anakinra. Nevertheless, at the final part of the ESC guidelines, the authors focus on the unmet needs namely the topics that require further research and evidence. Regarding RP topics needing further clarification include among others the complete decodification of the pathophysiology, the identification of risk factors associated with pericarditis recurrences, the adoption of medications beyond colchicine to prevent recurrences, the optimal medical treatment duration and tapering process, the indication for invasive techniques and their contribution to the etiological diagnosis in the realworld practice, and finally the adoption of individualized therapies according to the clinical scenarios [1]. More than 5 years after the publication of the 2015 guidelines the paper of Andreis A. et al. published in this issue of the International and Emergency Medicine is a valuable summary of the current status of knowledge of RP including the most recent piece of information on this challenging topic [6]. In specific this review paper addresses the * George Lazaros [email protected]