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

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

EFFUSIVE-CONSTRICTIVE PERICARDITIS: WHEN TO WAIT AND WHEN TO OPERATE? - Ushasi Saraswati (ACC 2025) - "Cardiac MRI showed severe pericardial inflammation.Decision-making: After multidisciplinary discussions, urgent pericardiectomy was deferred due to active inflammation, and the patient started on anakinra, later switched to rilonacept (aimed to manage inflammation), with outpatient monitoring. This case highlights the complexities of managing effusive-constrictive pericarditis with active inflammation. A comprehensive approach involving regular follow-ups and multidisciplinary collaboration is essential for effective management."

Cardiovascular • Congestive Heart Failure • Heart Failure • Inflammation • Respiratory Diseases • Ventricular Tachycardia

RECURRENT PERICARDITIS: WHEN IS IT TOO LATE TO OPERATE? - Ushasi Saraswati (ACC 2025) - "Initial treatment with ibuprofen led to recurrent chest pain and a large pericardial effusion requiring pericardiocentesis. She was started on prednisone (60 mg daily) and colchicine (0.6 mg twice daily) after pain recurrence during prednisone tapering. Cardiac MRI revealed severe pericardial late gadolinium enhancement (LGE).Decision-making: Azathioprine was initiated but later switched to anakinra due to persistent symptoms. Tapering anakinra led to recurrence, prompting rilonacept... Early intervention with biologic therapy is beneficial, but uncertainties about long-term risks underscore the need for evaluating pericardiectomy in patients with severe LGE. Further research is needed to identify which patients may benefit most from surgical intervention versus continued medical management."

Cardiovascular • Infectious Disease • Oncology • Pain

RELAPSE AFTER RELAPSE: A SIX-YEAR JOURNEY OF RECURRENT PERICARDITIS MANAGED WITH RILONACEPT, TREATED WITH IMAGE-GUIDED THERAPY - Tess Calcagno (ACC 2025) - "A tailored approach, including imaging and gradual tapering, is crucial in managing recurrent pericarditis."

Cardiovascular

CONTEMPORARY RECURRENT PERICARDITIS MANAGEMENT- REAL-WORLD EVIDENCE OF LIMITED CARDIAC MAGNETIC RESONANCE IMAGING PRIOR TO INITIATING RILONACEPT - Brittany Weber (ACC 2025) - "While CMR has utility amongst expert cardiologists in diagnosis, pericardial characterization, and monitoring, it was obtained in more clinically complex RP pts; rilonacept initiation was informed mostly by clinical criteria."

Clinical • HEOR • MRI • Real-world • Real-world evidence • Cardiovascular • Inflammation • IL1B

Rilonacept in Refractory Immune Checkpoint Inhibitor-Induced Recurrent Pericarditis: A Potential Treatment Option. (PubMed, JACC Case Rep) - "We present the case of a 63-year-old man with a history of metastatic melanoma treated with ipilimumab and nivolumab, who developed recurrent pericarditis complicated by cardiac tamponade on 2 separate occasions with 1 episode resulting in cardiac arrest. Despite ICI disruption and standard pericarditis treatment of a prolonged course of colchicine and prednisone, the patient experienced recurrent pericarditis. The introduction of rilonacept led to significant clinical improvement. This case demonstrates the potential effectiveness of rilonacept in managing ICI-induced recurrent pericarditis, especially when conventional treatment fails."

Checkpoint inhibition • Journal • Cardiovascular • Melanoma • Oncology • Solid Tumor

Current Drug Treatment for Acute and Recurrent Pericarditis. (PubMed, Drugs) - "Low- to moderate-dose glucocorticoids are reserved for patients with a first recurrence for which NSAIDs and colchicine failed and/or who have an autoimmune disorder, with a slow tapering. Interleukin-1 blockers-anakinra, rilonacept, and goflikicept-are used as a third-line option in patients who cannot come off glucocorticoids or as second-line therapy after NSAIDs and colchicine in patients with contraindications to glucocorticoids or in those with high-risk features (i.e., multiple episodes, markedly elevated inflammatory markers, or extensive abnormalities at pericardial imaging) in whom treatment with glucocorticoids is unlikely to succeed."

Journal • Review • Cardiovascular • Immunology

Recurrent Pericardial Effusion After Pediatric Kidney Transplant (NKF-SCM 2025) - "Here we present a novel case of recurrent pericardial effusion following a pediatric kidney transplant.Methods A 4-year-old boy with a LAMB2 mutation, congenital nephrotic syndrome and ESKD on hemodialysis received a living donor kidney transplant with basiliximab and steroid induction following a bradycardic arrest during intraoperative thymoglobulin administration. Kidney function rapidly normalized post-operatively and mycophenolate mofetil and tacrolimus maintenance therapy was started...His pericardial effusions continued to recur despite stepwise additions of colchicine and anakinra therapy...Anakinra was transitioned to rilonacept 3 for maintenance therapy and he has not had recurrence of pericardial effusion during eight months of follow-up.Results To our knowledge this is the first reported case of recurrent pericardial effusion after a pediatric kidney transplant in a patient receiving tacrolimus immunosuppression. Rapid effusion accumulation after cardiac..."

Clinical • Bone Marrow Transplantation • Cardiovascular • Glomerulonephritis • Infectious Disease • Nephrology • Pediatrics • Renal Disease • Rheumatology • Transplantation • LAMB2 • LAMC1

The Golden Card of Interleukin-1 Blockers in Systemic Inflammasomopathies of Childhood. (PubMed, Int J Mol Sci) - "Very few side effects have been reported with the long-term use of anakinra, rilonacept, or canakinumab, and their safety profile has been largely documented even in childhood. Further investigations into the role of inflammasomes in the pathogenesis of autoimmune conditions as well as brain degenerative or cardiovascular disorders can be expected, paving the way for precision medicine with benefits beyond inhibiting signaling by individual IL-1-family cytokines."

Journal • Review • Cardiovascular • Immunology • Inflammation • Pediatrics • Rare Diseases

TNF-a, IL-1, and JAK Inhibitors Associated with Increased Reporting Odds of Coccidioidomycosis: A Pharmacovigilance Study of FDA Adverse Event Reporting System (AAD 2025) - "The largest ROR was seen with rilonacept (ROR 143.02, 95% CI 35.58 - 574.86), followed by prednisone, infliximab, prednisolone, methylprednisolone, and betamethasone. The next highest agent was anakinra (ROR 12.8, 95% CI 5.74 - 28.56). Amongst the JAK inhibitors, baricitinib had the highest ROR followed by tofacitinib (ROR 7.51 (95% CI 1.87-30.06) vs 5.25 (95% CI 3.63 – 7.59))...Our study provides data indicating increased risk of coccidioidomycosis infections with the use of JAK and IL-1 inhibitors, along with corticosteroids. These findings could guide the use of screening tests for coccidioidomycosis in patients in endemic areas."

Adverse events • Infectious Disease • Respiratory Diseases • IL17A • IL23A • TNFA

The Role of the Advanced Practice Provider in a Pericardial Center of Excellence. (PubMed, Curr Cardiol Rep) - "The addition of Interleukin (IL-1) blockers or biologics (Rilonacept, Anakinra) in the last few years provides targeted therapy for these patients. Advanced Practice Providers play a vital role in a pericardial center with ordering the appropriate imaging and labs, handling medication titration, and providing patient education and continuity of care for these patients. They have been shown to decrease mortality, increase quality of care, and increase medication adherence."

Journal • Review • Cardiovascular

Deficiency of interleukin-1 receptor antagonist: A systematic review. (PubMed, Arch Rheumatol) - "There were no specific laboratory markers. Canakinumab was the drug of choice; however, glucocorticoids, rilonacept, and anakinra have been used."

Journal • Cardiovascular • Hematological Disorders • Infectious Disease • Inflammation • Musculoskeletal Diseases • Musculoskeletal Pain • Orthopedics • Pneumonia • Respiratory Diseases • Thrombosis • IL1RN

Pericarditis: state-of-the art and perspectives in 2025 (PubMed, Rev Med Suisse) - "Treatment combines non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine, with alternative options for recurrent and refractory forms such as corticosteroids, rilonacept and anakinra. The management strategy is adjusted according to patient characteristics and imaging: MRI plays a crucial role in assessing inflammation and monitoring treatment efficacy."

Journal • Cardiovascular • Inflammation

Kiniksa Pharmaceuticals Reports...Portfolio Execution (GlobeNewswire) - "ARCALYST net product revenue was $122.5 million and $417.0 million for the fourth quarter and full year 2024, respectively; Since launch, more than 2,850 prescribers have written ARCALYST prescriptions for recurrent pericarditis; As of the end of the fourth quarter of 2024, average total duration of ARCALYST therapy in recurrent pericarditis was approximately 27 months; As of the end of the fourth quarter of 2024, approximately 13% of the target 14,000 multiple-recurrence patients were actively on ARCALYST treatment....Kiniksa expects 2025 ARCALYST net product revenue of between $560 million and $580 million."

Commercial • Sales projection • Cardiovascular • Rare Diseases

Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome. (PubMed, Int J Mol Sci) - "IL-1 inhibitors (i.e., anakinra, canakinumab, and rilonacept) are prominent in the treatment of the disease, but the IL-6 receptor inhibitor tocilizumab and the Bruton's tyrosine kinase inhibitor ibrutinib are also promising alternatives. We diagnosed a patient who, to the best of our knowledge, represents the 748th documented case of this specific pathology. In the context of this patient, we would also like to draw attention to the potential pathogenic role of two novel gene mutations (variants of the MEFV gene "c.2084A>G" and the F2 gene "3'UTR c.*97G>A")."

Journal • Review • Dermatology • Developmental Disorders • Immunology • Inflammation • Musculoskeletal Pain • Pain • Urticaria • F2 • IL18 • IL6 • MYD88 • NLRP3

Advancement in the Treatment of Recurrent Pericarditis: Exploring Innovative Therapeutic Options. (PubMed, S D Med) - "Pericardiectomy serves as the ultimate resort. Notably, rilonacept emerges as a key player, specifically addressing cases resistant to colchicine and dependent on corticosteroids in recurrent pericarditis."

Journal • Review • Cardiovascular • Immunology

Assessing the Impact of Rilonacept on Lipid Profiles in Patients With Recurrent Pericarditis. (PubMed, JACC Adv) - No abstract available

Journal • Cardiovascular