Abstract

Background:Pericarditis is a common disease with significant morbidity (1). Idiopathic pericarditis, where an underlying cause cannot be identified, makes up for 80% of cases in the Western World (1). Up to 30% of these patients experience recurrence despite optimal treatment (2). Idiopathic recurrent pericarditis (IRP) is thought to represent an auto-inflammatory process rather than a reinfection (3). 2015 European Society of Cardiology (ESC) guidelines have outlined treatment of acute episodes and first recurrence with nonsteroidal anti-inflammatory drugs (NSAID), acetyl salicylic acid (ASS) and Colchicine as first line and Glucocorticoids (GC) as second line treatment (3). However GC treatment increases the risk of relapse, dependence and toxicity (2). Interleukin-1 (IL-1) inhibitors have been proposed as possible treatments in IRP (3, 4).Objectives:The aim of this case study is to outline our first experiences treating IRP with the IL-1 inhibitor anakinra in our Rheumatologic clinic.Methods:All patients referred to our department in 2018/2019 with pericarditis were physically seen in our outpatient clinic. All patients were screened for malignancy, infection or rheumatologic disease as possible cause by clinical measures. Following ESC guideline, patients who suffered either the third recurrence under optimal treatment or significant side effects or dependency from GC were considered for anakinra treatment. Daily injection of anakinra (100mg) were given continuously over at least three months with gradual tapering over at least three months afterwards. Physical emergency department contacts, days hospitalized, colchicine- and GC use, the year prior to Anakinra treatment was recorded retrospectively. During follow up the same data was prospectively recorded.Results:Over the course of two years 20 patients were referred to our clinic. All fulfilled ESC diagnostic criteria for pericarditis at index episode. In none of the patients could a rheumatologic, infectious or malignant cause be identified. 16 patients could be treated according to 2015 ESC guidelines with first or second line agents. Four patients were aligned to anakinra-treatment. Prior to referral, duration of symptoms was 5 - 120 months (mean 61 months). Further relevant patient- characteristics are outlined in Table 1.After initiation of anakinra patients were afterwards regularly followed up in scheduled visits every 3 months.Table 1.Characteristics of the four patients aligned to anakinra prior to anakinra-initiation.PatientNumber of recurrencesNumber emergency hospital contacts related to IRP the year prior to nakinraDays hospitalized related to IRP the year prior to anakinraGC dose prior to anakinraotherI2237.5 mgsteroidglaucomaII72410 mgIII472220 mgIV731220 mgFollow-up after start of anakinra was 6-15 months (mean 11.5 months). No patient was admitted to hospital or emergency department in that period. All four patients could taper and stop GC without recurrence. One patient experienced a mild relapse after discontinuing anakinra and was restarted on a low dose with complete remission. No patient had elevated CRP values at the end of follow-up and no patient experienced tamponade or clinical signs of constriction. No significant side effects were noted, no patient had to stop anakinra-treatment during follow up.Conclusion:Implementation of anakinra treatment in cases of complicated IRP was both secure and successful in our rheumatologic outpatient department. In our small sample we could confirm findings from bigger trials regarding effect- and side effect rates of anakinra treating IRP.

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