From Idiopathic to Autoinflammatory Pericarditis: Clinical characteristics, Inflammasome Pathways, Biomarkers and Therapeutic Advances

This Research Topic is dedicated to elucidating the transition of Idiopathic Recurrent Pericarditis (IRP) from a diagnosis of exclusion to a defined, targetable autoinflammatory syndrome. Pericarditis remains the most frequent form of pericardial disease, yet its recurrent form (RP) poses a substantial clinical and biological challenge. Historically, the majority of cases in developed nations were classified as "idiopathic," a label that reflects diagnostic inadequacy rather than a benign absence of pathology. This Research Topic seeks contributions that fundamentally redefine the pathophysiology, diagnosis, and management of this complex inflammatory disorder.The Centrality of Inflammasome Activation in RecurrenceThe conceptual shift classifying IRP as a polygenic IL-1 driven autoinflammatory disease fundamentally alters research directions. Acute pericarditis is now understood as a stereotypical inflammatory response to acute injury (viral illness or trauma), where Damage-Associated Molecular Patterns (DAMPs) or Pathogen-Associated Molecular Patterns (PAMPs) activate Pattern Recognition Receptors (PRRs).Crucially, the resulting immunological cascade is amplified by the activation of the NLRP3 inflammasome, triggering release of mature pro-inflammatory cytokines, (IL-1beta, IL-1alpha) and pyroptosis, perpetuating endothelial, pericardial damage and chronic disease progression.We invite research into the temporal activation of inflammasome components, specifically examining the potential involvement of other complexes such as NLRC4 and AIM2 in chronic disease progression or the transition to inflammatory constrictive pericarditis.The Imperative for Precision BiomarkersCurrent conventional inflammatory markers, such as C-reactive protein (CRP) and Erythrocyte Sedimentation Rate (ESR), are vital for confirming the acute diagnosis, but their utility in long-term prognosis and monitoring refractory recurrent pericarditis is limited. A substantial unmet need exists for reliable biomarkers, particularly for patients who present with clinical flares but remain CRP-negative.Research submissions focusing on advanced phenotyping are highly encouraged. This includes studies validating the prognostic role of molecular signatures, such as the expression profiles of IL-1, IL-6, IL-8, IL-10 and IL-12 in peripheral blood mononuclear cells (PBMCs) during relapse versus remission. Furthermore, submissions addressing the utility of novel markers are sought:1. Autoantibodies: Investigating anti-IL-1 Receptor Antagonist (IL-1RA) antibodies2. Chronic Inflammation Indicators: Exploring markers like Soluble Urokinase Plasminogen Activator Receptor (suPAR) to identify chronic low-grade inflammation that underlies challenging, non-overtly inflammatory phenotypes.3. Tissue Damage Markers: Further validation of the prognostic implications of cardiac troponins T and I.Therapeutic Advances and Future TargetsConventional therapy with high-dose NSAIDs, colchicine, and systemic corticosteroids fails in 10–15% of patients with refractory recurrence and poses toxicity risks with prolonged use. The targeted disruption of the IL-1 axis represents the most significant advance: IL-1 antagonists (Anakinra, Rilonacept, and Canakinumab) demonstrate high efficacy, favorable safety, and meaningful corticosteroid-sparing strategies. We invite studies comparing efficacy, safety, and long-term remission across these agents, including dual IL-1α/β blockade versus IL-1β-specific targeting. Studies identifying predictors of response or relapse and evaluating combination regimens (e.g., colchicine plus IL-1 blockade) are crucial to moving towards individualized treatment algorithms. Scope of SubmissionsWe invite submissions of high-quality Original Research Articles, Reviews, Mini-Reviews, Case Reports, and Perspective Articles addressing any aspect of the paradigm shift from idiopathic to autoinflammatory pericarditis, including, but not limited to:• Molecular mechanisms driving NLRP3, AIM2, and NLRC4 inflammasome activation in pericardial tissue.• The role of IL-1 family cytokines and pyroptosis in driving recurrent inflammation.• Clinical studies on precision phenotyping and biomarker validation (IE anti-IL-1RA antibodies, suPAR).• Comparative effectiveness and safety profiles of IL-1 antagonists and other biological therapies.• Genetic and epigenetic factors associated with autoinflammatory pericarditis susceptibility and therapeutic response. Topic editors: Emanuele Bizzi, Eva Reali, Angela Mauro, Lucia Trotta, Michiel Beyens Source: https://www.frontiersin.org/research-topics/75630/from-idiopathic-to-autoinflammatory-pericarditis-clinical-characteristics-inflammasome-pathways-biomarkers-and-therapeutic-advances