Rilonacept

Rilonacept is an interleukin-1 alpha (IL-1α) and interleukin-1 beta (IL-1β) cytokine trap. Rilonacept blocks IL-1 signaling by acting as a soluble decoy receptor that binds both IL-1α and IL-1β and prevents its interaction with cell surface receptors.

• ARCALYST (rilonacept) is an interleukin-1 blocker indicated for: Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older ( 1.1 , 14.1 ) Maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing 10 kg or more ( 1.2 , 14.2 ) Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and children 12 years and older ( 1.3 , 14.3 ) 1.1 Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome ARCALYST ® (rilonacept) is an interleukin-1 blocker indicated for the treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and pediatric patients 12 years and older. 1.2 Deficiency of IL-1 Receptor Antagonist ARCALYST is indicated for the maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing at least 10 kg.

• None. None.contraindicated

Administer by subcutaneous injection ( 2.1 ) CAPS, FCAS, MWS, and RP ( 2.2 ): Adults: – Loading dose: 320 mg, delivered as two 160 mg (2 mL) injections. – Maintenance dose: 160 mg (2 mL) injection once weekly. Pediatric patients 12 years to 17 years: – Loading dose: 4.4 mg/kg, up to a maximum of 320 mg, delivered as 1 or 2 injections (not to exceed 2 mL/injection). – Maintenance dose: 2.2 mg/kg, up to a maximum of 160 mg (2 mL) injection, once weekly. DIRA ( 2.3 ): Adults and pediatric patients weighing 10 kg or more: – 4.4 mg/kg up to a maximum of 320 mg, delivered as 1 or 2 injections (2 mL/injection) once weekly. 2.1 General Dosing Information ARCALYST is for subcutaneous use only. 2.2 Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome and Recurrent Pericarditis Adults : Initiate treatment with a loading dose of 320 mg delivered as two, 2-mL subcutaneous injections of 160 mg each, administered on the same day at two different injection sites. Continue dosing with a once-weekly injection of 160 mg administered as a single, 2-mL subcutaneous injection. Pediatric patients 12 years to 17 years : Initiate treatment with a loading dose of 4.4 mg/kg, up to a maximum dose of 320 mg, administered as one or two subcutaneous injections, not to exceed single-injection volume of 2 mL per injection site. If the initial dose is given as two injections, administer on the same day at two different sites.

Serious Infections : Serious, life-threatening infections have been reported in patients taking ARCALYST. Do not initiate treatment with ARCALYST in patients with active or chronic infections. Discontinue treatment if a patient develops a serious infection. ( 5.1 ) Hypersensitivity Reactions : If a hypersensitivity reaction occurs, discontinue administration of ARCALYST and initiate appropriate therapy. ( 5.3 ) Immunizations : Avoid live vaccines. Update recommended vaccinations prior to initiation of therapy per current guidelines. ( 5.5 ) 5.1 Serious Infections Interleukin-1 (IL-1) blockade may interfere with the immune response to infections. Treatment with another medication that works through inhibition of IL-1 has been associated with an increased risk of serious infections, and serious infections have been reported in patients taking ARCALYST [see Clinical Studies (14) ] . There was a greater incidence of infections in CAPS and RP patients on ARCALYST compared with placebo. In the controlled portion of the CAPS study [see Clinical Studies (14.1) ] , severe infection (bronchitis) was reported in one patient receiving ARCALYST. In an open-label extension study in CAPS, one patient developed bacterial meningitis and died [see Adverse Reactions (6.1) ] . In clinical studies, ARCALYST has not been administered concomitantly with tumor necrosis factor (TNF) inhibitors. An increased incidence of serious infections has been associated with administration of an IL-1 blocker in combination with TNF inhibitors.

The following clinically significant adverse reactions are described elsewhere in the labeling. Serious Infections [see Warnings and Precautions (5.1) ] Risk of Malignancy [see Warnings and Precautions (5.2) ] Hypersensitivity Reactions [see Warnings and Precautions (5.3) ] Lipid Profile Changes [see Warnings and Precautions (5.4) ] The most common adverse reactions reported by patients with CAPS and RP treated with ARCALYST are injection-site reactions and upper respiratory tract infections. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Kiniksa at 1-833-KINIKSA (1-833-546-4572) or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch . 6.1 Clinical Trial Experience Clinical trials are conducted under widely varying conditions and, as such, adverse reaction rates observed cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in clinical practice. The data described herein reflect exposure to ARCALYST in over 2,000 patients who received at least one dose, including approximately 1700 exposed to 160 mg or more, of whom 151 patients were exposed for at least 6 months and 111 patients for at least one year. These included patients with CAPS and RP, patients with other diseases, and healthy volunteers. CAPS Approximately 60 patients with CAPS were treated weekly with 160 mg of ARCALYST. The pivotal trial population included 47 patients with CAPS. These patients were between the ages of 22 and 78 years (average 51 years). Thirty-one patients were female and 16 were male. All of the patients were White/Caucasian.

Pregnancy: Based on animal data, may cause fetal harm. ( 8.1 ) 8.1 Pregnancy Risk Summary Rare pregnancy outcomes reported postmarketing and from clinical trials, with very limited use of ARCALYST in pregnant women, are insufficient to evaluate for a drug-associated risk of major birth defects, miscarriage or adverse maternal or fetal outcomes. There may be risks to the mother and fetus associated with Cryopyrin Associated Periodic Syndromes (CAPS) (see Clinical Considerations ) . In an animal reproduction study, subcutaneous administration of rilonacept to pregnant monkeys during the period of organogenesis was complicated by losses of drug exposure as the study progressed, due to anti-drug antibody formation at all doses, but a dose-related increase in exposure was still evident. There were no treatment-related effects on fetal survival or development of malformations with doses up to 11 times the maximum recommended human dose (MRHD). Increased incidences of lumbar ribs, a skeletal variation, were observed in fetuses at doses approximately 2 times the MRHD and higher that slightly exceeded incidences in both control animals and the historical control database ( see Data ).

Metabolism

The average trough levels of rilonacept were approximately 24 mcg/mL at steady state following weekly subcutaneous doses of 160 mg for up to 48 weeks in patients with CAPS. The steady state appeared to be reached by 6 weeks.

There have been no reports of overdose with ARCALYST. Maximum weekly doses of up to 320 mg have been administered subcutaneously for up to approximately 18 months in a small number of patients with CAPS and up to 6 months in patients with an unapproved indication in clinical trials without evidence of dose-limiting toxicities. In addition, ARCALYST given intravenously at doses up to 2000 mg monthly in another patient population for up to six months was tolerated without dose-limiting toxicities. The maximum amount of ARCALYST that can be safely administered has not been determined. In case of overdose, it is recommended that the patient be monitored for any signs or symptoms of adverse reactions or effects, and appropriate symptomatic treatment instituted immediately.

• Feb 27, 2008BLABLA125249Kiniksa Pharmaceuticals (uk), Ltd.

1. 1Injection Site Erythema11512%
2. 2Chest Pain10311%
3. 3Product Dose Omission Issue949.8%
4. 4Off Label Use808.3%
5. 5Fatigue798.2%
6. 6Injection Site Pain767.9%
7. 7Drug Ineffective707.3%
8. 8Pain677.0%
9. 9Pericarditis656.8%
10. 10Injection Site Pruritus596.1%
11. 11Injection Site Rash575.9%
12. 12Injection Site Reaction545.6%
13. 13Dyspnoea535.5%
14. 14Arthralgia515.3%
15. 15Nausea454.7%

The 10 most recently updated of 26 ClinicalTrials.gov registrations naming Rilonacept as an intervention. Registration is not evidence of efficacy or safety — reference crosswalk only.

• Rilonacept in Subjects With Cardiac Sarcoidosis
  Recruiting · Phase 2 · Interventional · 60 enrolled · Mayo Clinic
  NCT06660732updated 2025-08-15
• A Safety and Efficacy Study of Mycophenolate Mofetil and Rilonacept in Patients With Alcoholic Hepatitis
  Completed · Phase 2 · Interventional · 4 enrolled · Southern California Institute for Research and Education
  NCT01903798updated 2025-06-12
• Study to Assess the Efficacy and Safety of Rilonacept Treatment in Participants With Recurrent Pericarditis
  Completed · Phase 3 · Interventional · 86 enrolled · Kiniksa Pharmaceuticals (UK), Ltd.
  NCT03737110updated 2023-09-06
• A Pilot Study of KPL-914 in Recurrent Pericarditis
  Completed · Phase 2 · Interventional · 26 enrolled · Kiniksa Pharmaceuticals (UK), Ltd.
  NCT03980522updated 2021-05-27
• Inflammation in Chronic Kidney Disease and Cardiovascular Disease - The Role of Genetics and Interleukin-1 Receptor Antagonist (IL-1ra)
  Completed · Phase 2 · Interventional · 15 enrolled · VA Office of Research and Development
  NCT00897715updated 2019-10-01
• Cold Contact Urticaria Treatment With Rilonacept
  Completed · Phase 2 · Interventional · 20 enrolled · Charite University, Berlin, Germany
  NCT02171416updated 2018-07-17
• IL1-TRAP, Rilonacept, in Systemic Sclerosis
  Completed · Phase 1 · Phase 2 · Interventional · 24 enrolled · Boston University
  NCT01538719updated 2018-05-01
• Rilonacept for Deficiency of the Interleukin-1 Receptor Antagonist (DIRA)
  Completed · Phase 2 · Interventional · 6 enrolled · National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
  NCT01801449updated 2017-10-16
• Rilonacept for Treatment of Autoimmune Neurosensory Hearing Loss
  Unknown · Early phase 1 · Interventional · 10 enrolled · Stanley Cohen
  NCT02828033updated 2017-09-13
• Study of the Safety and Efficacy of Long-Term Rilonacept Treatment for the Prevention of Gout Flares
  Terminated · Phase 3 · Interventional · 220 enrolled · Regeneron Pharmaceuticals
  NCT01459796updated 2017-07-18

Frequently asked questions

How does Rilonacept work?

Rilonacept is an interleukin-1 alpha (IL-1α) and interleukin-1 beta (IL-1β) cytokine trap. Rilonacept blocks IL-1 signaling by acting as a soluble decoy receptor that binds both IL-1α and IL-1β and prevents its interaction with cell surface receptors.

What is Rilonacept used for?

According to FDA labeling, Rilonacept carries indications including: ARCALYST (rilonacept) is an interleukin-1 blocker indicated for: Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older ( 1.1 , 14.1 ) Maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing 10 kg or more ( 1.2 , 14.2 ) Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and children 12 years and older ( 1.3 , 14.3 ) 1.1 Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome ARCALYST ® (rilonacept) is an interleukin-1 blocker indicated for the treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and pediatric patients 12 years and older. 1.2 Deficiency of IL-1 Receptor Antagonist ARCALYST is indicated for the maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing at least 10 kg.. This is a reference summary of labeled uses, not medical advice or a treatment recommendation.

What class of drug is Rilonacept?

Rilonacept is classified as Interleukin inhibitors, Interleukin 1 Receptor Antagonists, Immunologic Activity Alteration.

What are the brand names for Rilonacept?

Rilonacept is marketed under brand names including Arcalyst.

What are the contraindications for Rilonacept?

Rilonacept labeling lists contraindications including: None. None.. Always consult the full prescribing information and a clinician.