rilonacept (Rx)

Brand and Other Names:Arcalyst
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 220mg/vial

Cryopyrin Associated Periodic Syndromes

Indicated for cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS)

Loading dose: 320 mg SC x 1 dose

Maintenance: 160 mg SC qWeek

Deficiency of Interleukin-1 Receptor Antagonist

Indicated for maintenance of remission of deficiency of interleukin-1 receptor antagonist (DIRA)

4.4 mg/kg (not to exceed 320 mg) SC once weekly

Pericarditis

Indicated to treat recurrent pericarditis and to reduce risk for recurrence

Loading dose: 320 mg SC x 1 dose

Maintenance: 160 mg SC qWeek

Dosage Modifications

Renal or hepatic impairment

  • No formal studies conducted

Dosing Considerations

DIRA

  • When switching from another IL-1 blocker, discontinue IL-1 blocker and begin rilonacept at next scheduled dose

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 220mg/vial

Cryopyrin Associated Periodic Syndromes

Indicated for cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS) in adults and children aged ≥12 yr

<12 years: Safety and efficacy not established

12-17 years

  • Loading dose: 4.4 mg/kg SC; not to exceed 320 mg total dose  
  • Maintenance: 2.2 mg/kg SC; not to exceed 160 mg/dose and 2 mL/injection

≥18 years

  • Loading dose: 320 mg SC once
  • Maintenance: 160 mg SC qWeek

Deficiency of Interleukin-1 Receptor Antagonist

Indicated for maintenance of remission of deficiency of interleukin-1 receptor antagonist (DIRA) in adults and pediatric patients weighing ≥10 kg

≥10 kg

  • 4.4 mg/kg not to exceed 320 mg) SC once weekly

Pericarditis

Indicated to treat recurrent pericarditis and to reduce risk for recurrence in adults and children aged ≥12 yr

<12 years: Safety and efficacy not established

12-17 years

  • Loading dose: 4.4 mg/kg SC; not to exceed 320 mg total dose  
  • Maintenance: 2.2 mg/kg SC; not to exceed 160 mg/dose and 2 mL/injection

≥18 years

  • Loading dose: 320 mg SC x 1 dose
  • Maintenance: 160 mg SC qWeek

Dosage Modifications

Renal or hepatic impairment

  • No formal studies conducted

Dosing Considerations

DIRA

  • When switching from another IL-1 blocker, discontinue IL-1 blocker and begin rilonacept at next scheduled dose
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Interactions

Interaction Checker

and rilonacept

No Results

     activity indicator 
    No Interactions Found
    Interactions Found

    Contraindicated

      Serious - Use Alternative

        Significant - Monitor Closely

          Minor

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            Adverse Effects

            >10%

            Injection site reactions (48%)

            Infections (34-48%)

            Upper respiratory tract infections (26%)

            1-10%

            Cough (9%)

            Hypoesthesia (9%)

            Sinusitis (9%)

            <1%

            Hypersensitivity reaction

            Neutropenia

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            Warnings

            Contraindications

            None

            Cautions

            Hypersensitivity reactions reported but rare; if hypersensitivity reaction occurs, discontinue treatment and initiate appropriate therapy

            Monitor for changes in lipid profiles and treat appropriately

            Immunosuppressants increase the risk of malignancies

            Increases in non-fasting lipid profile parameters reported; monitor patients’ lipid profiles and consider lipid-lowering therapies if needed, based on cardiovascular risk factors and current guidelines

            Infections

            • Interleukin-1 blockade may interfere with immune response to infections
            • Serious, life-threatening infections reported
            • Discontinue treatment with if serious infection develops
            • Do not initiate in patients with active or chronic infections

            Drug interaction overview

            • Live vaccines
              • Do not give live vaccines concurrently with rilonacept
              • Before initiation, patients should receive all recommended vaccinations
            • TNF-blocking agent and IL-1 blocking agent
              • Not recommended
              • Coadministration of drug that blocks IL-1 with a TNF-blocking agent associated with increased risk of serious infections and neutropenia
            • CYP450 substrates
              • Monitor therapeutic effect or drug concentration of CYP450 substrates with narrow therapeutic index; adjust dose accordingly
              • Increased levels of cytokines (eg, IL-1) suppress formation of CYP450 enzymes during chronic inflammation
              • Rilonacept may normalize the formation of these cytokines
              • May affect CYP450 substrates with narrow therapeutic indexes
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            Pregnancy & Lactation

            Pregnancy

            Data are insufficient on use in pregnant females to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes

            Animal data

            • Increased incidences of lumbar ribs, a skeletal variation, were observed in fetuses at doses ~2x the maximum recommended human dose (MRHD) and higher that slightly exceeded incidences in both control animals and the historical control database
            • Multiple fusion and absence of the ribs and thoracic vertebral bodies and arches in one fetus of the only pregnant monkey with drug exposure found during the later period of gestation associated with a dose ~6x the MRHD

            Disease-associated maternal and/or embryofetal risk

            Published data suggest that increased maternal levels of interleukin (IL)-1beta, which induces inflammation that occurs in CAPS, may be associated with preterm birth

            Lactation

            There is no information on presence in either human or animal milk, effects on breastfed infants, or effects on milk production

            Pregnancy Categories

            A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

            B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

            C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

            D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

            X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

            NA: Information not available.

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            Pharmacology

            Mechanism of Action

            Blocks interleukin-1alpha (IL-1alpha) and IL-1beta signaling by acting as a soluble decoy receptor that binds IL-1 alpha and beta and prevents interactions with cell surface receptors

            CAPS

            • Inflammation in CAPS is associated with mutations in the NLRP-3 gene which encodes the protein cryopyrin, an important component of inflammasome
            • Cryopyrin regulates the protease caspase-1 and controls the activation of IL-1beta; mutations in NLRP-3 result in an overactive inflammasome resulting in excessive release of activated IL-1beta that drives inflammation

            DIRA

            • Autoinflammatory, autosomal recessive disorder caused by loss of function mutations in the IL1RN gene, which encodes IL-1 receptor antagonist, resulting in unopposed signaling of the proinflammatory cytokines IL-1alpha and IL-1beta through the IL-1 receptor
            • Blocks IL-1beta signaling by acting as a soluble decoy receptor that binds IL-1beta and prevents its interaction with cell surface receptors
            • Also binds IL-1alpha and IL-1ra with reduced affinity

            Pericarditis

            • Interleukin-1 (IL-1) is a key cytokine that mediates the pathophysiology of many inflammatory processes, and it has been implicated as a causative factor in pericarditis
            • IL-1alpha and IL-1beta bind to the universally expressed cell surface receptor, IL-1 Receptor type-1, triggering a cascade of inflammatory mediators
            • Preformed IL-1alpha is released by damaged/inflamed pericardial cells and may contribute to the maintenance and amplification of inflammation via activation of the NLRP3 inflammasome, which then augments the inflammatory response by production of IL-1beta in a cascade amplification system

            Absorption

            Steady-state reached at 6 weeks

            Mean trough levels (steady-state) in pediatric patients with DIRA: 63.5-74 mg/mL

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            Administration

            SC Preparation

            Reconstitute with 2.3 mL sterile water for injection yielding 80 mg/mL solution

            Shake reconstituted vial for ~1 minute and then allow it to sit for 1 minute

            Use within 3 hr of reconstitution

            SC Administration

            SC use only

            Visually inspect reconstituted vial(s) for any discoloration or particulate matter; solution is viscous, clear, and colorless to pale yellow

            Withdraw dose, up to 160 mg (2 mL); with a new 27-gauge, 0.5-inch needle attached to a new 3-mL syringe

            For a 320-mg dose, deliver dose as 2 SC injections of 160-mg each on the same day at 2 different sites

            Do not administer more often than once weekly

            Single-use only vials; discard vial after withdrawal of drug

            Rotate injection sites (eg, abdomen, thigh, upper arm)

            Do not inject in area that is bruised, red, tender, or hard

            Storage

            Does not contain preservatives

            Unopened vials

            • Refrigerate at 2-8ºC (36-46ºF) in original container to protect from light
            • Do not use beyond stamped date on vial

            Reconstituted vials

            • Store at room temperature for up to 3 hr
            • Protect from light
            • Discard any unused portions
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            Formulary

            FormularyPatient Discounts

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            The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

            Tier Description
            1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
            2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
            3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
            4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            NC NOT COVERED – Drugs that are not covered by the plan.
            Code Definition
            PA Prior Authorization
            Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
            QL Quantity Limits
            Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
            ST Step Therapy
            Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
            OR Other Restrictions
            Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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            Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.