Arcalyst (rilonacept) / Regeneron, Kiniksa, Huadong Medicine - LARVOL DELTA

A Phase 2 Study of Rilonacept in Patients with Cardiac Sarcoidosis: Study Design (WASOG-AASOG 2025) - P2 | "Patients will be randomized 1:1 to receive either rilonacept (320 mg SC [loading dose] on day 1 followed by 160 mg SC weekly [maintenance dose]) added to standard therapy or non-biologic standard therapy (corticosteroids and steroid-sparing agents [e.g., azathioprine, methotrexate, mycophenolate, or leflunomide]) alone. REPAIR-CS will measure the efficacy of rilonacept added to standard therapy vs. non-biologic standard therapy alone in patients with CS, given the efficacy of rilonacept in certain diseases in which the IL-1 pathway is implicated."

Clinical • P2 data • Cardiovascular • Congestive Heart Failure • Heart Failure • Immunology • Infectious Disease • Sarcoidosis • CRP • IL1B

Comparisons of pericarditis patients on rilonacept with and without concomitant constrictive pericarditis: a case-control study (ESC-WCC 2025) - "Management of CP+ patients continues to be challenging, when rilonacept can be trialed to limit pericarditis flares, however pericardiectomy remains warranted in the majority. Further research is necessary to determine the optimal treatment strategy for CP+ pericarditis patients."

Clinical • Cardiovascular • Congestive Heart Failure • Heart Failure

Rilonacept as monotherapy for long-term recurrent pericarditis management: sustained disease control over three years (ESC-WCC 2025) - "Rilonacept as monotherapy provided recurrence-free persistence over the three-year trial duration when used continuously without interruption; pericarditis recurrences occurred only after interruptions. These findings support evidence-based use of rilonacept as monotherapy, minimizing the need for adjunctive treatments and reducing polypharmacy in long-term RP management. Further investigations are warranted to address how to assess pts as candidates for long-term treatment with rilonacept and when to cease rilonacept treatment."

Monotherapy • Cardiovascular • CRP • IL1B

Rilonacept reduces pericarditis recurrence risk: clinical outcomes from the RESONANCE patient registry (ESC-WCC 2025) - "This first report of real-world outcomes from RESONANCE shows that rilonacept-containing regimens for RP as 1st, 2nd, or 3rd line therapy reduced recurrence risk over long- term (>2 year) treatment, supporting prolonged management during RP natural history."

Clinical • Clinical data • Cardiovascular • IL1B

IL-1 Pathway Inhibition in Recurrent Pericarditis Management: Real-World Adoption of Corticosteroid Sparing in RESONANCE. (PubMed, JACC Adv) - P | "In RESONANCE, IL-1 pathway inhibition increasingly replaced corticosteroids as second-line therapy. Most patients starting corticosteroids transitioned to IL-1 pathway inhibition; few transitioned from second-line IL-1 pathway inhibition to long-term corticosteroids. These findings may inform treatment algorithms and patient-provider decision-making."

Journal • Real-world evidence • Cardiovascular • Inflammation • Pediatrics

A Comprehensive Clinical Evaluation of Rilonacept in the Treatment of Recurrent Pericarditis: A Systematic Review. (PubMed, J Inflamm Res) - "Rilonacept is effective, tolerable, innovative, and suitable in the treatment of RP, while also showing promise in improving its cost-effectiveness and accessibility. Further comparative and cost-effectiveness studies are needed to fully define the therapeutic role of rilonacept in the RP treatment paradigm."

Journal • Review • Cardiovascular • Inflammation • Pain • Rare Diseases • CRP

Desmoplakin Cardiomyopathy Presenting With Recurrent Myopericarditis Responsive to Interleukin-1 Blockade. (PubMed, JACC Case Rep) - "Nine months after initiating rilonacept, the patient remained asymptomatic, had been off prednisone for 8 months, and demonstrated improved exercise tolerance and normalized troponin I levels. This case highlights the potential benefit of interleukin-1 blockade in managing DSP-related recurrent myopericarditis."

Journal • Cardiomyopathy • Cardiovascular • Congestive Heart Failure • Crohn's disease • Gastroenterology • Gene Therapies • Genetic Disorders • Heart Failure • Immunology • Inflammatory Bowel Disease • Pain • KCNQ1OT1

Kiniksa Pharmaceuticals Reports Second Quarter 2025 Financial Results… (GlobeNewswire) - "ARCALYST net product revenue was $156.8 million for the second quarter of 2025; Since launch, more than 3,475 prescribers have written ARCALYST prescriptions for recurrent pericarditis; As of the end of the second quarter of 2025, average total duration of ARCALYST therapy in recurrent pericarditis was approximately 30 months....Kiniksa expects 2025 ARCALYST net product revenue of between $625 million and $640 million, compared to prior guidance of between $590 million and $605 million; Kiniksa expects its current operating plan to remain cash flow positive on an annual basis."

Sales • Sales projection • Immunology • Inflammation • Rare Diseases

Schnitzler syndrome - a rare cause of chronic urticaria. Case report. (PubMed, Oxf Med Case Reports) - "Treatment options include highly effective interleukin-1 blockade therapies such as anakinra, canakinumab, and rilonacept. This case emphasizes the importance of a thorough differential diagnosis of chronic urticaria and encourages clinicians to participate in the SchS database for improved recognition and management."

Journal • Chronic Spontaneous Urticaria • Dermatology • Immunology • Inflammation • Monoclonal Gammopathy • Urticaria • Vasculitis • CRP

Systematic Review of Adverse Events of IL-1 and IL-6 Inhibitor Use in Pediatrics. (PubMed, J Pediatr Pharmacol Ther) - "Risks of biological use should be considered alongside the immunosuppressive benefits when prescribing IL-1 and IL-6 inhibitors in the pediatric population. Few data were available on long-term follow-up of these patients."

Adverse events • Journal • Infectious Disease • Musculoskeletal Pain • Pain • Pediatrics

Expanding the Patient Pool for Rilonacept. (PubMed, JACC Case Rep) - "The RHAPSODY trial (Study to Assess the Efficacy and Safety of Rilonacept Treatment in Participants With Recurrent Pericarditis) showed rilonacept, an interleukin-1 cytokine trap, to be an effective therapeutic option for patients with pericarditis recurrence. This case series demonstrates the utility and challenges of rilonacept use in the types of patients excluded from RHAPSODY."

Journal • Cardiovascular

Real-world data and Mendelian randomization analysis in assessing adverse reactions of rilonacept. (PubMed, Int J Clin Pharm) - "Rilonacept is effective for certain inflammatory conditions, but careful monitoring for adverse reactions, particularly involving the immune system, skin, and cardiac issues, is essential."

Journal • Real-world evidence • Cardiovascular • Dermatology • Immunology • Infectious Disease • Inflammation • Novel Coronavirus Disease • Pain • Urticaria • IL1RN

Difficult-to-treat recurrent pericarditis after SARS-CoV-2 vaccination. (PubMed, Int J Cardiol) - "Pericarditis following SARS-CoV-2 vaccination can evolve into a recurrent, difficult-to-manage inflammatory condition. Effective treatment may require IL-1 blockade to disrupt the autoinflammatory cycle. Prompt recognition and early escalation of therapy are essential to reduce morbidity and prevent complications."

Journal • Cardiovascular • Infectious Disease • Novel Coronavirus Disease • Pain • Respiratory Diseases

Running into trouble: exercise-exacerbated recurrent pericarditis-a case report. (PubMed, Eur Heart J Case Rep) - "Despite colchicine and NSAIDs, symptoms recurred, and MRI confirmed active pericarditis with late gadolinium enhancement of the pericardium. Given the refractory nature of his disease, biologic therapy with rilonacept was initiated. This case highlights the association between high-intensity exercise and recurrent pericarditis, underscoring the need for individualized treatment strategies, including biologics. It contributes to the understanding of refractory cases and exercise-induced inflammation."

Journal • Cardiovascular • Inflammation • Musculoskeletal Pain • Pain

Resolution of Colchicine-Resistant, Corticosteroid-Dependent Acute Idiopathic Recurrent Pericarditis With Rilonacept: A Case Report. (PubMed, Cureus) - "Eight months after the initial diagnosis, she experienced rising inflammatory markers and intermittent fevers despite treatment with ibuprofen and colchicine. Her condition progressed to corticosteroid dependence and marginal pericardial calcification, identified via an echocardiogram eight days after recurrent symptoms began. Symptom resolution and inflammation control were achieved with rilonacept, showing sustained success at a 12-month follow-up."

Journal • Cardiovascular • Diabetes • Infectious Disease • Oncology • Osteoporosis • Pain • Rheumatology

Comprehensive Clinical Analysis of Rilonacept in the Treatment of Cryopyrin-Associated Periodic Syndromes: A Systematic Review. (PubMed, J Multidiscip Healthc) - "It shows high innovativeness and acceptable suitability, with the potential for improved cost-effectiveness and accessibility. We anticipate that the effective treatment of CAPS with rilonacept will encourage further clinical and fundamental research, offering valuable insights for the treatment of other autoinflammatory diseases."

Journal • Review • Inflammation • Rare Diseases • CRP

A REVIEW OF DE NOVO INFLAMMATORY BOWEL DISEASE IN PATIENTS ON IL-1 INHIBITOR THERAPY FOR RHEUMATOLOGIC DISEASES (DDW 2025) - "Introduction : Interleukin-1 (IL-1) inhibitors, including anakinra, canakinumab, and rilonacept are increasingly utilized in the management of various rheumatologic disorders due to their ability to target proinflammatory cytokines IL-1 alpha and IL-1 beta. Clinicians should be aware of the potential complications of this class of drug. Furthermore, these reports are ripe for further study, including formal review of individual reports in an effort to seek pharmacovigilance."

Clinical • Review • Gastroenterology • Gastrointestinal Disorder • Hematological Disorders • Immunology • Inflammation • Inflammatory Bowel Disease • Rheumatology • IL1A • IL1B

A Case of Recurrent Hemophagocytic Lymphohistiocytosis-like Episodes in a Patient with XIAP Deficiency (CIS 2025) - "Concerned he may have developed antibodies to canakinumab, he was trialed on anakinra, which resolved his fever and symptoms. He was discharged on rilonacept and has remained fever- and symptom-free since. Discussions regarding HSCT remain ongoing. This case highlights the importance of targeted pharmaceutical therapies, such as anti-IL-1, in managing complex inflammatory conditions like XLP2."

Clinical • Anemia • Bone Marrow Transplantation • Crohn's disease • Gastroenterology • Gastrointestinal Disorder • Hematological Disorders • Hemophagocytic lymphohistiocytosis • Immunology • Infectious Disease • Inflammation • Inflammatory Bowel Disease • Musculoskeletal Pain • Primary Immunodeficiency • Rare Diseases • CD163 • CD4 • CD8 • CXCL9 • IL18 • IL1B • LAMP1 • XIAP

Kiniksa Pharmaceuticals Reports First Quarter 2025 Financial Results and Recent Portfolio Execution (GlobeNewswire) - "ARCALYST (rilonacept) Q1 2025 net product revenue of $137.8 million, representing 75% year-over-year growth; ARCALYST 2025 expected net product revenue increased to $590 - $605 million."

Sales • Cardiovascular • Immunology • Inflammation

Tumor Necrosis Factor Receptor-Associated Periodic Syndrome: Genetics, Autoinflammation, and Recurrent Pericarditis. (PubMed, JACC Case Rep) - "RP is often labeled idiopathic, but autoinflammatory diseases such as TRAPS should be considered in such cases on the basis of the clinical presentation. Interleukin-1 inhibitors have emerged as promising treatments for RP, with radical pericardiectomy being considered as a last resort."

Journal • Cardiovascular • Immunology • Oncology • Pain • TNFA

Rilonacept Use in Recurrent Pericarditis After Combined Heart-Liver Transplantation (ISHLT 2025) - "We describe the use of rilonacept in a heart-liver transplant recipient with recurrent pericarditis despite prednisone and colchicine.Case Report A 22 year-old male with hypoplastic left heart syndrome post-Fontan surgery and Fontan-associated liver disease underwent heart-liver transplantation with pulmonary artery reconstruction...His immunosuppression regimen at the time included tacrolimus with trough of 10-12 ng/mL, mycophenolate mofetil 1000 mg twice daily and prednisone 7.5 mg daily...Summary Rilonacept is an option for recurrent pericarditis in heart transplant patients who do not respond to more conventional therapies. Further data are needed regarding the management of immunosuppression and risk of infection during rilonacept therapy."

Cardiovascular • Gastrointestinal Disorder • Hepatology • Infectious Disease • Pain • Pulmonary Embolism • Respiratory Diseases • Solid Organ Transplantation • Transplantation • CRP

TO FIND THE EXIT AT A DIFFICULT CROSSROADS: MANAGEMENT OF INFECTIVE ENDOCARDITIS (IE) REQUIRING REDO STERNOTOMY, IN A PATIENT ON RILONACEPT THERAPY FOR RECURRENT POST-PERICARDIOTOMY PERICARDITIS - Bianca Honnekeri (ACC 2025) - "Planning redo sternotomy in patients with IE with demonstrated response to rilonacept for previous post-pericardiotomy syndrome involves complex planning due to lack of high quality data on optimal postoperative rilonacept therapy resumption strategies, balanced against the risk of postoperative pericarditis flare. In the absence of evidence-based guidelines, management should be individualized based on shared, multidisciplinary decision-making."

Clinical • Cardiovascular • Heart Failure • Infectious Disease • Inflammation • Pain

INTRACTABLE PERICARDITIS ASSOCIATED WITH FIBROSING MEDIASTINITIS DESPITE BIOLOGICS AND RADICAL PERICARDIECTOMY - Sakthi Surya Prakash (ACC 2025) - "Initial treatment involved triple therapy (colchicine, prednisone, ibuprofen). However, attempts to taper steroids led to recurrences, and quadruple therapy with azathioprine, anakinra, and rilonacept was tried sequentially... Chronic recurrent pericarditis could be a rare complication of FM. Even after radical pericardiectomy, CMR guided therapy with rilonacept may be necessary to maintain steroid free remission."

Cardiovascular • Fibrosis • Inflammation

TURNING THE TIDE ON REFRACTORY PERICARDITIS WITH IL-1 INHIBITORS - Jean Michel Saad (ACC 2025) - "Despite trials of colchicine, celecoxib, ibuprofen and steroids, he remained with recurrent symptoms. Post-ablation pericarditis poses significant management challenges, with traditional treatments sometimes inadequate for refractory cases. Integrating IL-1 inhibitors into treatment strategies could enhance outcomes for these patients, highlighting the need for continued exploration and adoption of these advanced therapies."

Cardiomyopathy • Cardiovascular • Inflammation • Pain

A CASE OF RECURRENT PERICARDITIS AFTER INCOMPLETE PARTIAL PERICARDIECTOMY - RILONACEPT OR REDO- PERICARDIECTOMY? - Sakthi Surya Prakash (ACC 2025) - "It was treated with drainage, partial pericardiectomy and colchicine, which was switched to prednisone due to diarrhea. Recurrent pericarditis can occur with partial pericardiectomy. In such cases, CMR can be used to initiate biologic therapy and monitor progress, reserving redo-pericardiectomy for refractory cases."

Clinical • Cardiovascular • Inflammation • Pain