This is a US, non-interventional, registry for pediatric and adult patients with RP. This patient registry is an organized data collection system that uses observational methods to collect uniform data on a patient population that is followed over time. There will be two types of data collection: retrospective via chart review and prospective including Patient Reported Outcomes (PROs). The registry will launch when the first patient is enrolled, and it is currently planned to collect data for approximately 500 patients over a minimum period of 5 years.
To characterize the clinical course, impact, management, and treatment of adults and children with RP, in order to:
Active RP is defined, for the purpose of this registry, as patients who are currently under the care of a physician for treatment of RP, currently on treatment for RP, and have had an episode (recurrence of typical pericarditis pain associated with supportive objective evidence of pericarditis) in the last 3 years prior to enrollment. Once included in the registry, these patients will have both retrospective and prospective follow-up and data collection (hybrid design).
Inactive RP is defined, for the purpose of this registry, as patients who have had a diagnosis of RP, have not had an episode for 3 years and have not been prescribed any treatment for RP in the last 3 years, prior to enrolling in the registry. Once included in the registry, data collected from these patients will be retrospective only.
The target ratio of enrollment between the two cohorts will be as follows: 90% of active RP patients and 10% of inactive RP patients. The intent is to focus the effort on capturing prospective, longitudinal data. However, retrospective data collection is included for active and inactive cohorts, to enable various comparisons between historical and contemporaneous data-sets.
Patients with active RP
Patients with inactive RP
Recurrent pericarditis (RP) can be caused by a variety of different factors, post-surgery, post Percutaneous Transluminal Coronary Angioplasty (PTCA), post ablation, and in developed countries, viral infection. However, approximately 80% of adults and 70% of children with RP have idiopathic RP.¹ The underlying pathogenesis of RP remains unclear, although a growing body of evidence suggests that abnormal immune responses play a role. While the adaptive immune system is involved in autoimmune disorders that manifest with pericarditis (systemic lupus erythematosus or rheumatoid arthritis), the innate immune system, including nucleotide-binding domain-like receptor protein 3 (NLRP3) inflammasome [which stimulates secretion of interleukin 1 (IL-1)] signaling pathway, is often the major effector in autoinflammatory disorders, such as isolated pericarditis.²⁻³
Diagnosis of a pericarditis episode requires the presence of at least two of the four following criteria: precordial chest pain that is worse with inspiration and when supine, characteristic ST segment elevation and PR deviation on electrocardiogram (ECG), a pericardial friction rub, and a pericardial effusion that is more than trivial.⁴⁻⁵ Elevations of certain markers of inflammation or evidence of pericardial inflammation by an imaging technique are used as supportive findings.
Recurrent pericarditis is a serious autoinflammatory disease with major short-term and long-term challenges for clinicians and patients alike. Now, a more complete clinical picture is emerging thanks to a new understanding of IL-1–mediated pathophysiology.
1. Tombetti E., Giani T, Brucato A, Cimaz R. Recurrent Pericarditis in Children and Adolescents. 2019; Frontiers in Pediatrics (7)
2. Baskar S, Klein AL, Zeft A. The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review. Cardiol Res Pract. 2016;2016:7840724.
3. Brucato A, Imazio M, Gattorno M, et al. Effect of Anakinra on Recurrent Pericarditis Among Patients With Colchicine Resistance and Corticosteroid Dependence: The AIRTRIP Randomized Clinical Trial. JAMA. 2016;316(18):1906-1912.
4. Imazio M, Gaita F, LeWinter M. Evaluation and Treatment of Pericarditis: A Systematic Review. JAMA. 2015;314(14):1498-1506.
5. Lazaros G, Antonatou K and Vassilopoulos D. The Therapeutic Role of Interleukin-1 Inhibition in Idiopathic Recurrent Pericarditis: Current Evidence and Future Challenges. Frontiers in Medicine. 2017, 4:78