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Aim. To evaluate the long-term safety and efficacy of goflkicept treatment in patients with idiopathic recurrent pericarditis (IRP). Materials and methods. This report presents the interim analysis of an ongoing open-label extension (OLE) clinical trial of goflkicept in patients with IRP (NCT 05673902), as a continuation of the core study (NCT04692766). The study assessed the frequency of pericarditis recurrence, time to recurrence after 12 and 60 weeks of goflkicept therapy, changes in C-reactive protein level, chest pain intensity, pericardial effusion size, and adverse events (AEs). Results. All patients remained in clinical-laboratory remission during the 60 weeks of goflkicept treatment. The recurrence frequency was 31.3% (5/16) after 60 weeks and 90% (9/10) after 12 weeks of goflkicept treatment (p0.001). A total of 64 AEs were reported in 16 patients (94.1%), mostly of mild to moderate severity. The most common AEs were infections, occurring in 11 patients (64.7%). Nine serious adverse events were reported in 5 patients, none of which were considered drug-related. There were no deaths. Conclusion. Long-term goflkicept therapy resulted in a significant reduction in the risk of recurrence and prolonged remission without an increase in adverse events.

Pericarditis is one of the most common forms of inflammatory diseases of the cardiovascular system, diseases that are observed in cardiology, immunology and rheumatology. Anti-inflammatory therapy is the mainstay of treatment for pericarditis. Low recommendations for the diagnosis and treatment of pericarditis for about 10 years, and in 2025, guide their next update. Professor Massimo Imasio, who is a member of the Working Group on Diagnosis and Various Diseases of the Pericardium of the European Society of Cardiology (ESC, European Society of Cardiology) and the Association of Cardiothoracic Surgery (EACTS, European Association of Cardiothoracic Surgery) 2015 and Chairman of the 2025 ESC Working Guidelines on Myocarditis and Pericarditis, gave an interview regarding the most pressing issues in the diagnosis and treatment of pericarditis.

Pericarditis, a clinical syndrome characterized by inflammation and thickening of the pericardium, is one of the most common forms of inflammatory diseases of the cardiovascular system. The most common and severe complication of acute pericarditis is idiopathic recurrent pericarditis (IRP), which has a poor prognosis associated with the risk of cardiac tamponade and constrictive pericarditis. The pathogenesis of pericarditis is associated with a complex interaction of environmental factors, genetic predisposition, and pathological activation of innate and acquired immunity. Autoinflammatory mechanisms associated with hyperproduction of interleukin (IL) 1 attract particular attention. Standard therapy for pericarditis includes non-steroidal antiinflammatory drugs, colchicine, glucocorticoids, and immunosuppressive drugs. A new direction in the pharmacotherapy of pericarditis is associated with the use of Anakinra (a recombinant non-glycosylated analog of an IL-1 receptor antagonist), which blocks the signaling of IL-1β and IL-1α. The materials of numerous studies are summarized, indicating that Anakinra is an effective drug for the treatment of patients with IRI who are resistant to standard therapy. It is assumed that the wider use of Anakinra, especially in the early stages of pericarditis, will not only improve the prognosis, but also be important for the identification of the autoinflammatory phenotype of IRI and the development of personalized therapy programs.

Aim: To evaluate the long-term safety and efficacy of goflkicept treatment in patients with idiopathic recurrent pericarditis (IRP)., Materials and Methods: This report presents the interim analysis of an ongoing open-label extension (OLE) clinical trial of goflkicept in patients with IRP (NCT05673902), as a continuation of the core study (NCT04692766). The study assessed the frequency of pericarditis recurrence, time to recurrence after 12 and 60 weeks of goflkicept therapy, changes in C-reactive protein level, chest pain intensity, pericardial effusion size, and adverse events (AEs)., Results: All patients remained in clinical-laboratory remission during the 60 weeks of goflkicept treatment. The recurrence frequency was 31.3% (5/16) after 60 weeks and 90% (9/10) after 12 weeks of goflkicept treatment (p<0.001). A total of 64 AEs were reported in 16 patients (94.1%), mostly of mild to moderate severity. The most common AEs were infections, occurring in 11 patients (64.7%). Nine serious adverse events were reported in 5 patients, none of which were considered drug-related. There were no deaths., Conclusion: Long-term goflkicept therapy resulted in a significant reduction in the risk of recurrence and prolonged remission without an increase in adverse events.

Idiopathic recurrent pericarditis (IRP) and adult-onset Still's disease (AOSD) are polygenic autoinflammatory diseases, in the pathogenesis of which pro-inflammatory cytokines from the interleukin-1 superfamily play a central role.Aim. To compare serum concentrations of proinflammatory cytokines and glycosylated ferritin (GF) in patients with IRP and AOSD during an exacerbation.Material and methods. The study included 15 patients with AOSD, 15 — IRP. The diagnosis of AOSD was established using the Yamaguchi criteria (1992). IRP was diagnosed in accordance with the 2015 European Society of Cardiology on the diagnosis and management of pericardial diseases. Blood sampling from all patients was carried out during the recurrence period prior to the anti-inflammatory therapy initiation. The serum levels of interleukin-1β (IL-1β), interleukin-6 (IL-6), interleukin-18 (IL-18), procalcitonin, total ferritin and GF was assessed. The results obtained were compared with levels of biochemical parameters, high-sensitivity C-reactive protein (CRP), as well as with white blood cell (WBC) and neutrophil counts.Results. The median age in the AOSD group was 28 years, and the IRP — 55 years. An increase WBC count >10*109/L was detected in 10 and 9 patients in the AOSD and IRP groups, respectively. The concentration of CRP was increased in all patients and did not differ in the study groups (p=0,836).The highest values of ferritin and GF levels were found in the AOSD group (1416 ng/ml vs 408 ng/ml, p=0,008) and (12% vs 33,9%, p=0,067), respectively. In both groups, increased concentrations of IL-6 and IL-18 were determined. In the AOSD group, the concentration of IL-18 was higher than in the IRP group (2114 pg/ml vs 161,5 pg/ml, p

Polygenic autoinflammatory diseases (AIDs), such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease, Kawasaki disease, idiopathic recurrent pericarditis (IRP), Behçet’s Syndrome, Crystal-induced arthropatihes such as gout or Calcium pyrophosphate deposition disease are characterized by the overexpression of inflammasome-associated genes, leading to a dysregulation of the innate immune response. The IL-1 cytokine family (IL-1α, IL-1β, IL-1Ra, IL-18, IL-36Ra, IL-36α, IL-37, IL-36β, IL-36g, IL-38, IL-33) was defined to be principally responsible for the inflammatory nature of polygenic AIDs. Several clinical trials were initiated, and IL-1 blockade has been proven to cause a rapid reduction of clinical symptoms and normalization of laboratory parameters in the majority of cases. Randomized, placebo-controlled, clinical trials, together with registry-based clinical trials and open-label, retrospective and prospective observational studies, supported the efficacy and safety of IL-1 inhibitors in the treatment of polygenic AIDs. Most of the current data are focused on the therapeutic use of anakinra, an IL-1 receptor antagonist, canakinumab, an anti-IL-1β monoclonal antibody, and rilonacept, a soluble decoy receptor. However, other promising agents, such as gevokizumab, IL-1β blocking monoclonal antibody, tadekinig alfa, a human recombinant IL-18-binding protein, and tranilast, an analog of a tryptophan metabolite, are currently being tested. Anakinra, canakinumab and rilonacept caused impressive improvements in both systemic and musculoskeletal symptoms. Furthermore, the anti-IL-1 therapy allowed corticosteroid tapering and, in some cases, even withdrawal. This article reviews the current IL-1 inhibitors and the results of all clinical trials in which they have been tested for the management of broad spectrum of polygenic AIDs.

Glucocorticoid (GC)-dependent, colchicine-resistant idiopathic recurrent pericarditis (IRP) remains a clinical challenge. We assessed for the first time the efficacy and safety of hydroxychloroquine (HCQ) in IRP. This is a post hoc analysis of prospectively collected data of 15 patients with refractory to standard therapy (colchicine plus either GC or anakinra) IRP (≥3 recurrences, disease duration ≥12 months and inability to wean off treatment) treated with HCQ (400 mg/day). These patients were matched 1:1 for age, sex, and treatment type to IRP patients receiving standard-of-care treatment (control group, n = 15). Pericarditis recurrence, the time to 1st flare, the % of patients able to achieve a ≥50% reduction of baseline GC dose and the % reduction of GC dose, were compared between groups. HCQ did not reduce pericarditis recurrence risk as almost all patients (n = 29) but one in the HCQ group (14/15) relapsed during follow-up. However, HCQ treatment was associated with an increased median time of flare-free survival (increase by 4 weeks compared to controls) and reduced hazard ratio for flare in survival analysis (HR = 0.36, 95% CI 0.16–0.77, p = 0.009). HCQ was also associated with a higher proportion of patients obtaining a ≥50% dose reduction of GCs (33.3% vs. 0% in the control group, p = 0.037) and reduced GC dose (HCQ: −43.5% vs. control: −4.5%, p < 0.001). No differences in CRP levels at flare was detected (p = 0.615). In this prospective study, HCQ depicted a GC-sparing effect and an increased flare-free survival period in patients with colchicine resistant GC-dependent IRP. • Hydroxychloroquine (HCQ) appears as a novel, steroid-sparing agent for patients with colchicine-resistant, steroid-dependent pericarditis • HCQ was associated with a significant reduction in the glucocorticoid dose (by almost a half) and extension of the flare-free survival (by 4 weeks) • Given HCQ excellent safety profile, low cost and steroid-sparing effect, these preliminary findings need to be confirmed in a larger trial [ABSTRACT FROM AUTHOR]

In developed countries, more than 80% of cases of acute pericarditis remain without an established diagnosis after a conventional and standard diagnostic approach. These cases are generally labelled as 'idiopathic', i.e. without a known cause. This lack of information is a matter of concern for both patients and clinicians. Some years ago, this term reflected the state of the art of scientific knowledge on the topic. Advances have changed this point of view, in light of available molecular techniques like polymerase chain reaction able to identify viral cardiotropic agents in pericardial fluid and biopsies. Furthermore, the remarkable efficacy of interleukin-1 antagonists, a therapy targeting the innate immune response, suggests clinical and pathogenic similarity between a proportion of patients with idiopathic recurrent pericarditis and classical autoinflammatory diseases. So, it seems useful to discuss the pros and cons of using the term "idiopathic" in light of the new knowledge. [ABSTRACT FROM AUTHOR]

Idiopathic recurrent pericarditis ( IRP) can be challenging to treat. Even after guideline-directed first-line treatment consisting of aspirin ( ASA) or a nonsteroidal antiinflammatory drug ( NSAID) in combination with colchicine therapy, recurrences still occur in greater than 20% of patients. Many patients then require treatment with long-term corticosteroids, which is not a favorable option due to their short- and long-term adverse effects. Because it is theorized that the pathophysiology of IRP may possess autoimmune sequelae, the use of immunotherapy for the treatment of IRP has emerged. In this review, we describe the literature associated with immunotherapy used to treat IRP in an adult population as well as provide an overview of the safety and monitoring parameters for each agent. The most common immunotherapies used after patients have had multiple recurrences of IRP are anakinra, intravenous immunoglobulin ( IVIG), and azathioprine. In most cases, these immunotherapies are adjunctive therapy, with the goal of tapering and discontinuing immunosuppressive corticosteroids. After reviewing the data, anakinra resulted in more patients discontinuing corticosteroids and prevented further recurrences of pericarditis. IVIG resulted in symptom resolution and no further recurrences in most of the patients. Azathioprine was associated with more than half of patients becoming recurrence free; however, many patients required a restart of corticosteroids due to recurrence. Clinicians should be aware of the adverse effects of immunotherapy, ranging from mild gastrointestinal events to risk of infection and serious blood dyscrasias that may require diligent monitoring. The use of immunotherapy for the treatment of adults with IRP should be restricted to patients who have multiple recurrences. Ideally, immunotherapy would be adjunctive to first-line combination therapy with ASA/ NSAID plus colchicine, with the goal of tapering and discontinuing immunosuppressive corticosteroids. Furthermore, clinicians should consider cost, drug-drug and drug-disease interactions, and safety, as well as the quality of the retrospective evidence before considering any immunotherapy. [ABSTRACT FROM AUTHOR]

Idiopathic recurrent pericarditis occurs in 20 to 50% of patients after the first episode of acute pericarditis. Etiology and pathogenesis are controversial; however, nowadays it is known more about autoimmune and inflammatory processes that could be the cause, giving us new guidelines and treatment options. The approach corresponds to acute pericarditis; however, the definitive diagnosis is made by exclusion of all known causes and is usually done retrospectively. First line of treatment are the non steroidal analgesics with aspirin and colchicine, leaving as second line steroids for highly selected cases and as a new option the receptor antagonists of IL-1 (anakinra) as a specific treatment. [ABSTRACT FROM AUTHOR]

Polygenic autoinflammatory diseases (AIDs), such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease, Kawasaki disease, idiopathic recurrent pericarditis (IRP), Behçet's Syndrome, Crystal-induced arthropatihes such as gout or Calcium pyrophosphate deposition disease are characterized by the overexpression of inflammasome-associated genes, leading to a dysregulation of the innate immune response. The IL-1 cytokine family (IL-1α, IL-1β, IL-1Ra, IL-18, IL-36Ra, IL-36α, IL-37, IL-36β, IL-36g, IL-38, IL-33) was defined to be principally responsible for the inflammatory nature of polygenic AIDs. Several clinical trials were initiated, and IL-1 blockade has been proven to cause a rapid reduction of clinical symptoms and normalization of laboratory parameters in the majority of cases. Randomized, placebo-controlled, clinical trials, together with registry-based clinical trials and open-label, retrospective and prospective observational studies, supported the efficacy and safety of IL-1 inhibitors in the treatment of polygenic AIDs. Most of the current data are focused on the therapeutic use of anakinra, an IL-1 receptor antagonist, canakinumab, an anti-IL-1β monoclonal antibody, and rilonacept, a soluble decoy receptor. However, other promising agents, such as gevokizumab, IL-1β blocking monoclonal antibody, tadekinig alfa, a human recombinant IL-18-binding protein, and tranilast, an analog of a tryptophan metabolite, are currently being tested. Anakinra, canakinumab and rilonacept caused impressive improvements in both systemic and musculoskeletal symptoms. Furthermore, the anti-IL-1 therapy allowed corticosteroid tapering and, in some cases, even withdrawal. This article reviews the current IL-1 inhibitors and the results of all clinical trials in which they have been tested for the management of broad spectrum of polygenic AIDs. [ABSTRACT FROM AUTHOR]

Heart transplant (HTx) recipients are at increased risk of pericardial disease. Idiopathic recurrent pericarditis has not been previously described following HTx. We describe a 35‐year‐old male who was admitted with pericarditis and moderate pericardial effusion 10 months after HTx. Two weeks before his admission, his prednisone had been tapered off. A thorough infectious workup and endomyocardial biopsy was unrevealing. He was started on colchicine with the addition of tapering prednisone regimen of 40 mg daily due to unresolved pain. Over the next several years, he had three recurrent episodes of pericarditis requiring re‐initiation of prednisone with extensive investigations negative for rejection, autoimmune, and infectious causes. Cardiac MRI confirmed pericardial inflammation. Due to his recurrent course and inability to wean off prednisone, anakinra, an IL‐1 receptor antagonist, was started at 100 mg sc daily. This allowed successful discontinuation of prednisone. He is now 34 months post‐transplant without recurrence on anakinra and colchicine maintenance. Due to the overlap between idiopathic recurrent pericarditis and auto‐inflammatory diseases, there is growing evidence for utilizing IL‐1 receptor antagonists in this condition. While pericarditis is common in the HTx population, this is the first report of successful use of an IL‐1 receptor blocker for pericarditis in this population. [ABSTRACT FROM AUTHOR]

Conventional algorithm for treatment of pericarditis and prevention of recurrences consists of non-steroid anti-inflammatory drugs and/or colchicine, followed by corticosteroids in resistant patients. Anakinra has emerged as a promising and safe treatment modality for steroid-dependent idiopathic recurrent pericarditis. However, the efficacy and safety of canakinumab, another anti-interleukin-1 agent, has not been assessed up to date. Herein, we present development of an anaphylactic reaction due to anakinra and a successful subsequent treatment with canakinumab for the first time in the literature.