rilonacept

REMS

Cryopyrin-associated periodic syndromes, including familial cold autoinflammatory syndrome and Muckle-Wells syndrome.
Maintenance of remission in deficiency of interleukin-1 receptor antagonist.
Treatment and reduction in risk of recurrent pericarditis.

Action ⬆ ⬇

Modulates cryopyrin by blocking interleukin-1 beta (IL-1β) preventing its interaction with surface receptors.

Therapeutic effects:

Decreased inflammatory manifestations of cryopyrin-associated periodic syndromes and deficiency of interleukin-1 receptor antagonist.
Reduction in episodes of and decreased pain associated with pericarditis.

Pharmacokinetics ⬆ ⬇

Absorption: Absorbed following SUBQ administration.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: Unknown.

Time/Action Profile ⬆ ⬇

(improvement in symptoms)

ROUTE	ONSET	PEAK	DURATION
SUBQ	within several days	unknown	unknown

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Active or chronic infections.

Use Cautiously in:

At risk of infection;
OB: Safety not established in pregnancy;
Lactation: Safety not established in breastfeeding;
Pedi: Safety and effectiveness not established in children <12 yr (cryopyrin-associated periodic syndromes or pericarditis) or <10 kg (deficiency of interleukin-1 receptor antagonist).

Adv. Reactions/Side Effects ⬆ ⬇

Local: injection site reactions

Metab: changes in lipid profile

Neuro: hypoesthesia

Resp: upper respiratory tract infections, cough

Misc: hypersensitivity reactions, INFECTION

Interactions ⬆ ⬇

Drug-drug:

May ↓ the antibody response to and ↑ adverse reactions from live vaccines; vaccination should take place prior to initiation of treatment.
TNF inhibitors may ↑ risk of serious infections; avoid concurrent use.
CYP450 enzyme substrates, especially those with narrow therapeutic indices such as warfarin, should be monitored carefully, as enzyme activity may ↑ (normalize) as a result of treatment.

Route/Dosage ⬆ ⬇

Cryopyrin-Associated Periodic Syndromes, Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, and Recurrent Pericarditis

SC (Adults ): 320 mg (given as two 160-mg injections) initially, then 160 mg (given as one injection) once weekly.
SC (Children 12–17 yr): 4.4 mg/kg (max = 320 mg) (given as one or two injections, with max of 160 mg per injection site) initially, then 2.2 mg/kg (max = 160 mg) (given as one injection) once weekly.

Deficiency of Interleukin-1 Receptor Antagonist

SC (Adults ): 320 mg (given as two 160-mg injections) once weekly.
SC (Children ≥10 kg): 4.4 mg/kg (max = 320 mg) (given as one or two injections, with max of 160 mg per injection site) once weekly.

Availability ⬆ ⬇

Lyophilized powder for injection: 220 mg/vial

Assessment ⬆ ⬇

Assess for signs of infections. Discontinue therapy if serious infection occurs. Do not administer to patients with active or chronic infections.
Assess for signs of hypersensitivity reactions; institute symptomatic therapy.
.

Lab Test Considerations:

Monitor lipid profiles every 2–3 mo during therapy. May cause ↑ total cholesterol, HDL, LDL, and triglycerides. Consider lipid lowering therapies as needed.

Implementation ⬆ ⬇

Administer all recommended adult and pediatric vaccinations, including pneumococcal vaccine and inactivated influenza vaccine, prior to starting rilonacept therapy.
Administer first dose under supervision of health care professional.
Loading dose in adults is given as two 2 mL injections on the same day in 2 different injection sites. In pediatric patients 1 or 2 injections may be used. Do not administer more than once weekly or more then 2 mL/injection site.
Reconstitute with 2.3 mL of sterile water for injection using a 27-gauge ½-inch needle for a concentration of 80 mg/mL. Discard needle and syringe after reconstitution. Shake vial for 1 min and allow to sit for 1 min. Solution is viscous, clear, colorless to pale yellow. Do not administer solutions that are discolored, contain particulate matter, or are passed the expiration date. Withdraw recommended dose up to 2 mL with new 27-gauge ½-inch needle. Vials are single use; discard unused solution. Rilonacept must be refrigerated and protected from light. Reconstituted solution is stable for 3 hr at room temperature.
SC: Inject SUBQ into abdomen (avoid area within 2 inches of navel), thigh or upper arm by pinching skin and injecting at a 90° (45° angle for small children or persons with little fat). May require 30 seconds to inject entire dose. Pull needle out of skin and hold gauze over site for several seconds. Rotate sites and avoid sites that are bruised, red, tender or hard.

Patient/Family Teaching ⬆ ⬇

Instruct patient or family on correct technique for preparing and administering injection, and disposing of equipment. If a dose is missed, administer up to the day before next scheduled dose. Take next dose at regularly scheduled time. Advise patient to read Patient Information prior to starting therapy and with each Rx refill in case of new information.
Advise patient to notify health care professional immediately if signs of infection (fever, cough, flu-like symptoms, open sores) occur during therapy.
Instruct patient to notify health care professional or seek emergency care if signs of allergic reaction (rash, swollen face, difficulty breathing) occur.
Advise patient that injection site reactions (erythema, swelling pruritus, bruising, inflammation, pain, edema, dermatitis, urticaria, vesicles, warmth, and hemorrhage) may occur; usually resolve in 1–2 days. Notify health care professional if reaction is persistent.
Instruct patient to consult health care professional prior to taking other Rx, OTC, or herbal products.
Rep: Advise female patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

Evaluation/Desired Outcomes ⬆ ⬇

Decrease in symptoms (rash, joint pain, fever, tiredness) and decrease in and normalization of serum amyloid A and C-reactive protein levels in cryopyrin-associated periodic syndromes and deficiency of interleukin-1 receptor antagonist.
Reduction in episodes of and decreased pain associated with pericarditis.

US Brand Names ⬆ ⬇

Code ⬆

NDC Code

61755- Regeneron Pharmaceuticals, Inc.
- 61755-931-
  - 61755-931-00- 1000 mL in 1 BOTTLE (61755-931-00)

65044- Jubilant HollisterStier LLC
- 65044-7168-
  - 65044-7168-1- 2 mL in 1 VIAL, SINGLE-USE (65044-7168-1)

73604- Kiniksa Pharmaceuticals (UK), Ltd.
- 73604-914- Arcalyst
  - 73604-914-01- 1 VIAL, SINGLE-USE in 1 CARTON (73604-914-01) / 2 mL in 1 VIAL, SINGLE-USE

73604- Kiniksa Pharmaceuticals (UK), Ltd.
- 73604-914- Arcalyst
  - 73604-914-04- 4 VIAL, SINGLE-USE in 1 CARTON (73604-914-04) / 2 mL in 1 VIAL, SINGLE-USE

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇