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.

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.

Availability ⬆ ⬇

Lyophilized powder for injection: 220 mg/vial

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.

US Brand Names ⬆ ⬇

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.

Classifications ⬆ ⬇

Therapeutic Classification: orphan drugs

Pharmacologic Classification: fusion proteins, interleukin antagonists

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

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.

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Indications ⬇