Rilonacept

What is Rilonacept?

• Rilonacept (Arcalyst) is an interleukin-1 blocker used to treat cryopyrin-associated periodic syndromes, including familial cold autoinflammatory syndrome, and Muckle–Wells syndrome; deficiency of interleukin-1 receptor antagonist; and recurrent pericarditis.

What are the uses of this medicine?

• Rilonacept (Arcalyst) is a prescription medicine called an interleukin-1 (IL-1) blocker ,used to treat:
• adults and children 12 years and older with Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS)
• maintain control of symptoms of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and children weighing at least 22 pounds (10 kg).
• Recurrent Pericarditis (RP) and reduce the risk of recurrence in adults and children 12 years and older.

How does this medicine work?

• Rilonacept (ril on’ a sept) is a recombinant fusion protein which includes the extracellular portion of the human IL-1 receptor and the IL-1 receptor accessory protein fused with the Fc portion of human IgG1.
• Rilonacept binds and inactivates IL-1, acting as an “IL-1 trap”.
• IL-1 is a key proinflammatory cytokine that is a powerful inducer of fever and inflammation.
• Excessive production of IL-1 or lack of its inactivation is believed to play a major role in many autoinflammatory conditions and particularly in cold induced autoinflammatory conditions such as cryopyrin associated periodic syndromes (CAPS).
• IL-1 may also play an important role in inflammatory arthritis and familial Mediterranean fever.
• In controlled trials and open label studies, rilonacept has been shown to improve symptoms and laboratory abnormalities associated CAPS and, to a lesser extent, in patients with gouty arthritis, juvenile idiopathic arthritis, familial Mediterranean fever and in rare forms of autoinflammatory conditions such as Schnitzler syndrome.

Who Should Not Use this medicine ?

• This medicine have no usage limitations.

What drug interactions can this medicine cause?

• Patients not take medicines that block tumor necrosis factor (TNF), such as Enbrel® (etanercept), Humira® (adalimumab), or Remicade® (infliximab), while you are taking ARCALYST.
• You or your child should also not take other medicines that block interleukin-1 (IL-1), such as Kineret® (anakinra), while taking ARCALYST.
• Taking ARCALYST with any of these medicines may increase your or your child's risk of getting a serious infection.

Before starting treatment with ARCALYST tell the healthcare provider if you or your child:

• think you have an infection.
• are being treated for an infection.
• have signs of an infection, such as fever, cough, or flu-like symptoms.
• have any open sores on your body.
• have a history of infections that keep coming back.
• have asthma. People with asthma may have an increased risk of infection.
• have diabetes or an immune system problem. People with these problems have a higher chance for infections.
• have tuberculosis (TB), or if you have been in close contact with someone who has had tuberculosis.
• have or have had HIV, hepatitis B, or hepatitis C.
• take other medicines that affect your immune system.
• Corticosteroids

Is this medicine FDA approved?

• Rilonacept was given an orphan drug designation by the U.S. Food and Drug Administration (FDA) and is used for the treatment of cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome, Muckle–Wells syndrome.
• Rilonacept is the first drug approved by the FDA to treat recurrent pericarditis.
• Rilonacept was approved for medical use in the United States in February 2008.

How should this medicine be used?

Recommended dosage: Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome and Recurrent Pericarditis: 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.

Deficiency of IL-1 Receptor Antagonist: 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.

Administration:

• Take or give ARCALYST exactly as prescribed by the healthcare provider.
• ARCALYST is given by injection under the skin (subcutaneous injection) 1 time each week.
• ARCALYST should not be given more than 1 time each week.
• The healthcare provider will tell you how much ARCALYST to take or give your child, and show you how to prepare and give the injection.
• Do not try to give ARCALYST injections until you are sure that you understand how to prepare and inject your dose. Call the healthcare provider or pharmacist if you have any questions, or if you would like more training.
• If you or your child take too much ARCALYST, call the healthcare provider or go to the nearest hospital emergency room right away.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As injection: 220 mg of lyophilized powder in a single-dose vial for reconstitution.

This medicine is available in fallowing brand namesː

• ARCALYST

What side effects can this medication cause?

The most common side effects of this medicine include: In people with CAPS and RP, the most common side effects of ARCALYST include:

• Injection-site reactions including: pain, redness, swelling, itching, bruising, lumps, inflammation, skin rash, blisters, warmth, and bleeding at the injection site.
• Upper respiratory tract infections
• Joint and muscle aches in RP

In people with DIRA, the most common side effects of ARCALYST include:

• Upper respiratory infection
• Rash
• Ear infection
• Sore throat
• Runny nose

ARCALYST can cause serious side effects, including:

• affect your or your child's immune system
• Risk of Cancer
• Allergic Reaction
• Changes in your blood cholesterol and triglycerides (lipids)

What special precautions should I follow?

• 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.
• Hypersensitivity reactions associated with ARCALYST occurred in clinical trials. If a hypersensitivity reaction occurs, discontinue administration of ARCALYST and initiate appropriate therapy.
• Avoid live vaccines. Update recommended vaccinations prior to initiation of therapy per current guidelines. Since ARCALYST may interfere with normal immune response to new antigens, vaccines may not be effective in patients receiving ARCALYST.
• The impact of treatment with ARCALYST on the development of malignancies is not known. Treatment with immunosuppressants, including ARCALYST, may result in an increase in the risk of malignancies.
• Rilonacept has had limited clinical use and has yet to be linked to cases of clinically apparent, acute liver injury.

What to do in case of emergency/overdose?

Symptoms of overdosage may include:

• There have been no reports of overdose with ARCALYST.
• 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.

Management of overdosage:

• 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.

Can this medicine be used in pregnancy?

• It is not known if ARCALYST will harm your unborn child.
• Tell the healthcare provider right away if you become pregnant while taking ARCALYST.

Can this medicine be used in children?

• It is not known if ARCALYST is safe and effective in children under 12 years of age.
• It is not known if ARCALYST is safe and effective in children with DIRA weighing less than 22 pounds (10 kg).

What are the active and inactive ingredients in this medicine?

• Active ingredient: rilonacept.
• Inactive ingredients: glycine, histidine, L-arginine, polyethylene glycol 3350 and sucrose.

Who manufactures and distributes this medicine?

Manufactured by:

• Kiniksa Pharmaceuticals (UK), Ltd.
• London, UK W1S 4PZ

What should I know about storage and disposal of this medication?

• Keep ARCALYST in the carton it comes in to protect from light.
• Store ARCALYST in the refrigerator from 36°F to 46°F (2°C to 8°C). Call your pharmacy if you have any questions.
• Refrigerated ARCALYST can be used until the expiration date printed on the vial and carton.
• ARCALYST may be kept at room temperature after mixing with Sterile Water for Injection, USP, and should be used within 3 hours of mixing. Keep ARCALYST away from light.

antirheumatic agents

• gold preparations
  • auranofin, gold salts, gold sodium thiomalate

• interleukin receptor antagonists
  • anakinra, canakinumab, rilonacept, sarilumab, tocilizumab

• major immunosuppressive agents (also used in transplant medicine)
  • cyclosporine, mycophenylate, sirolimus, tacrolimus

• monoclonal antibodies
  • adalimumab, canakinumab, certolizumab, golimumab, infliximab, rituximab, sarilumab, tocilizumab, ustekinumab

• thiopurines
  • azathioprine, mercaptopurine, thioguanine

• tumor necrosis factor antagonists
  • adalimumab, certolizumab, etanercept, golimumab, infliximab

• tyrosine (janus) kinase inhibitors
  • baricitinib, tofacitinib

• miscellaneous
  • abatacept, flavocoxid, hydroxychloroquine, leflunomide, methotrexate, penicillamine

• Dailymed label info on Rilonacept
• FDA Rilonacept

Rilonacept Resources
Latest articles Most recent articles Ongoing trials	Wikipedia

Rilonacept Resources
Latest articles Most recent articles Ongoing trials	Wikipedia