Clinical Burden and Unmet Need in Recurrent Pericarditis: A Systematic Literature Review

Klein, Allan L.; Cremer, Paul; Kontzias, Apostolos; Furqan, Muhammad; Forsythe, Anna; Crotty, Christopher P.; Lim-Watson, M.; Magestro, Matthew

doi:10.1097/crd.0000000000000356

Cited by 9 publications

(7 citation statements)

References 59 publications

(145 reference statements)

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“…Approximately 40% of patients with RP included in this study experienced multiple recurrences, which is consistent with prior estimates (range: 24-55%) [3,11]. In some of these patients, recurrences may cause life-threatening complications such as constrictive pericarditis and cardiac tamponade [4]; a recent systematic literature review of real-world studies showed that cardiac tamponade and constrictive pericarditis occur in 12.7% and 1.84% of patients with RP (i.e., with or without multiple recurrences), respectively [18]. Furthermore, disease duration exceeded 3 years for nearly half of patients with multiple recurrences.…”

Section: Discussionsupporting

confidence: 84%

“…Conventional treatments fail to provide adequate symptom control and prevention of future recurrences for many patients with pericarditis. Sustained NSAIDs and systemic steroids are complicated by serious side effects [4,12,18,30,31]. Until recently, there were no FDA-approved treatments for pericarditis; rilonacept was recently approved for treatment of recurrent pericarditis and reduction in risk of recurrence.…”

Section: Discussionmentioning

confidence: 99%

“…Until recently, there were no FDA-approved treatments for pericarditis; rilonacept was recently approved for treatment of recurrent pericarditis and reduction in risk of recurrence. The off-label use of therapies and lack of US treatment guidelines lead to inconsistencies in patient treatment and disease management strategies and adversely affect clinical outcomes [5,18].…”

Section: Discussionmentioning

confidence: 99%

“…While colchicine in combination with NSAIDs demonstrated efficacy among patients with multiple recurrences [17], a significant proportion of patients treated with this regimen will subsequently experience RP. Consequently, patients with multiple recurrences may suffer from a higher disease burden and use more healthcare services [4,18]. Thus, there is clearly a high unmet need for new treatments among patients with multiple pericarditis recurrences [5].…”

Section: Introductionmentioning

confidence: 99%

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Disease and Economic Burden Associated with Recurrent Pericarditis in a Privately Insured United States Population

et al. 2021

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Introduction: Approximately 30% of patients with a first acute pericarditis episode experience a recurrence B 18 months; * 15% experience multiple recurrences. This study assessed the recurrence and economic burden among patients with multiple recurrences. Methods: Adults with idiopathic pericarditis were identified in the OptumHealth Care Solutions, Inc., database (2007Inc., database ( -2017. Recurrent pericarditis (RP) was defined as C 2 episodes of care separated by [ 28 days; multiple recurrences were defined as C 2 recurrences. Results: Among 944 patients with RP, 375 (39.7%) experienced multiple recurrences and were propensity score-matched 1:1 to 375 patients without recurrence. Among patients with multiple recurrences, median disease duration (time from first episode to end of last recurrence, confirmed by a 1.5-year recurrencefree period) was 2.84 years. The multiple recurrences cohort had higher rates of hospitalizations per-patient-per-month (PPPM) than the no recurrence cohort (rate ratio [95% confidence interval (CI)] = 2.22 [1.35-3.65]). Mean total healthcare costs were significantly higher in the multiple recurrences versus no recurrence cohort

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Disease and Economic Burden Associated with Recurrent Pericarditis in a Privately Insured United States Population

et al. 2021

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“…Idiopathic recurrent pericarditis (IRP) is a poorly understood sequela of acute pericarditis that impacts significantly on quality of life. 1 , 2 , 3 IRP management remains challenging, with no US guidelines currently in place to aid clinicians and, although studies have demonstrated that corticosteroids are associated with increased recurrence risk, their use in IRP remains widespread. 1 , 2 , 3 , 4 Consequently, there is a need to understand the mechanisms driving IRP, to guide management that minimizes treatment‐associated harm.…”

Section: Tablementioning

confidence: 99%

Pericarditis and Autoinflammation: A Clinical and Genetic Analysis of Patients With Idiopathic Recurrent Pericarditis and Monogenic Autoinflammatory Diseases at a National Referral Center

Peet

Rowczenio

Omoyinmi

et al. 2022

JAHA

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Background Idiopathic recurrent pericarditis (IRP) is an orphan disease that carries significant morbidity, partly driven by corticosteroid dependence. Innate immune modulators, colchicine and anti‐interleukin‐1 agents, pioneered in monogenic autoinflammatory diseases, have demonstrated remarkable efficacy in trials, suggesting that autoinflammation may contribute to IRP. This study characterizes the phenotype of patients with IRP and monogenic autoinflammatory diseases, and establishes whether autoinflammatory disease genes are associated with IRP. Methods and Results We retrospectively analyzed the medical records of patients with IRP (n=136) and monogenic autoinflammatory diseases (n=1910) attending a national center (London, UK) between 2000 and 2021. We examined 4 genes ( MEFV, MVK, NLRP3, TNFRSF1A ) by next‐generation sequencing in 128 patients with IRP and compared the frequency of rare deleterious variants to controls obtained from the Genome Aggregation Database. In this cohort of patients with IRP, corticosteroid dependence was common (39/136, 28.7%) and was associated with chronic pain (adjusted odds ratio 2.8 [95% CI, 1.3–6.5], P =0.012). IRP frequently manifested with systemic inflammation (raised C‐reactive protein [121/136, 89.0%] and extrapericardial effusions [68/136, 50.0%]). Pericarditis was observed in all examined monogenic autoinflammatory diseases (0.4%–3.7% of cases). Rare deleterious MEFV variants were more frequent in IRP than in ancestry‐matched controls (allele frequency 9/200 versus 2932/129 200, P =0.040). Conclusions Pericarditis is a feature of interleukin‐1 driven monogenic autoinflammatory diseases and IRP is associated with variants in MEFV , a gene involved in interleukin‐1β processing. We also found that corticosteroid dependence in IRP is associated with chronic noninflammatory pain. Together these data implicate autoinflammation in IRP and support reducing reliance on corticosteroids in its management.

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A Brief Overview of Recurrent Pericarditis Management and the Potential of Rilonacept as a New Therapeutic Option

Gupta

Kaul

Velazquez

et al. 2021

Am J Cardiovasc Drugs

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Clinical Burden and Unmet Need in Recurrent Pericarditis: A Systematic Literature Review

Abstract: Supplemental Digital Content is available in the text.

Cited by 9 publications

References 59 publications

Disease and Economic Burden Associated with Recurrent Pericarditis in a Privately Insured United States Population

Disease and Economic Burden Associated with Recurrent Pericarditis in a Privately Insured United States Population

Pericarditis and Autoinflammation: A Clinical and Genetic Analysis of Patients With Idiopathic Recurrent Pericarditis and Monogenic Autoinflammatory Diseases at a National Referral Center

A Brief Overview of Recurrent Pericarditis Management and the Potential of Rilonacept as a New Therapeutic Option

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