Usual Adult Dose for Pericarditis

Initial dose: 320 mg (given as two 2 mL subcutaneous injections of 160 mg each) on the same day at 2 different injection sites
Maintenance dose: 160 mg subcutaneously once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence

Usual Adult Dose for Familial Cold Autoinflammatory Syndrome

Initial dose: 320 mg (given as two 2 mL subcutaneous injections of 160 mg each) on the same day at 2 different injection sites
Maintenance dose: 160 mg subcutaneously once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence

Usual Adult Dose for Muckle Wells Syndrome

Initial dose: 320 mg (given as two 2 mL subcutaneous injections of 160 mg each) on the same day at 2 different injection sites
Maintenance dose: 160 mg subcutaneously once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence

Usual Adult Dose for Interleukin-1 Receptor Antagonist Deficiency

320 mg (given as two 2 mL subcutaneous injections of 160 mg each) on the same day at 2 different injection sites

Comments:

• This drug should not be given more often than once a week.

Use: For maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA)

Usual Pediatric Dose for Pericarditis

12 years and older:
Initial dose: 4.4 mg/kg (up to a maximum of 320 mg) given as 1 or 2 subcutaneous injections with a maximum single-injection volume of 2 mL (if the initial dose is given as 2 injections, they should be given on the same day at 2 different sites)
Maintenance dose: 2.2 mg/kg (up to a maximum of 160 mg) given as a single subcutaneous injection up to 2 mL once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in children 12 years and older
• For maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in children 12 years and older

Usual Pediatric Dose for Familial Cold Autoinflammatory Syndrome

12 years and older:
Initial dose: 4.4 mg/kg (up to a maximum of 320 mg) given as 1 or 2 subcutaneous injections with a maximum single-injection volume of 2 mL (if the initial dose is given as 2 injections, they should be given on the same day at 2 different sites)
Maintenance dose: 2.2 mg/kg (up to a maximum of 160 mg) given as a single subcutaneous injection up to 2 mL once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in children 12 years and older
• For maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in children 12 years and older

Usual Pediatric Dose for Muckle Wells Syndrome

12 years and older:
Initial dose: 4.4 mg/kg (up to a maximum of 320 mg) given as 1 or 2 subcutaneous injections with a maximum single-injection volume of 2 mL (if the initial dose is given as 2 injections, they should be given on the same day at 2 different sites)
Maintenance dose: 2.2 mg/kg (up to a maximum of 160 mg) given as a single subcutaneous injection up to 2 mL once a week

Comments:

• This drug should not be given more often than once a week.

Uses:

• For treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS) in children 12 years and older
• For maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA)
• For treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in children 12 years and older

Usual Pediatric Dose for Interleukin-1 Receptor Antagonist Deficiency

Weight 10 kg or greater:
4.4 mg/kg as 1 or 2 subcutaneous injections once a week (if given as 2 injections, administer both the same day, at different sites)
Maximum dose: 320 mg

Comments:

• This drug should not be given more often than once a week.

Use: For maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in pediatric patients weighing at least 10 kg

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

Dose modification is not required based on advanced age or gender.

Precautions

CONTRAINDICATIONS:

• None

Safety and efficacy have not been established in patients younger than 12 years for Cryopyrin-Associated Periodic Syndromes (CAPS) and Recurrent Pericarditis (RP). Safety and effectiveness in pediatric patients with DIRA weighing 10 kg or more have been
established [see Adverse Reactions (6.1) and Clinical Studies (14.2)].Safety and effectiveness of
ARCALYST have not been established in pediatric patients weighing less than 10 kg for
maintenance of remission of DIRA.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• This drug is for subcutaneous use only.
• Each vial should be used for a single dose only.
• Prior to injection, the reconstituted solution should be carefully inspected for any discoloration or particulate matter. If there is discoloration or particulate matter in the solution, the product in that vial should not be used.
• Sites for subcutaneous injection (e.g., abdomen, thigh, upper arm) should be rotated.
• Injections should never be made at sites that are bruised, red, tender, or hard.
• The first injection should be performed under the supervision of a healthcare professional.
• If a patient or caregiver is to administer this drug, he/she should be instructed on aseptic reconstitution of the product and injection technique.
• The ability to inject subcutaneously should be assessed to ensure proper administration, including rotation of injection sites.

Storage requirements:

• The lyophilized product should be stored refrigerated at 2C to 8C (36F to 46F) in the original carton to protect it from light.
• Do not use beyond the date stamped on the label.
• After reconstitution, this drug may be kept at room temperature, should be protected from light, and should be used within 3 hours of reconstitution.
• This drug does not contain preservatives; therefore, unused portions should be discarded.

Reconstitution/preparation techniques:

• Reconstitution with 2.3 mL of preservative-free Sterile Water for Injection is required prior to subcutaneous administration of this drug.
• The reconstituted solution should be viscous, clear, colorless to pale yellow, and essentially free from particulates.
• See the manufacturer product information for reconstitution instructions.

Patient advice:

• Read the patient information that comes with this drug before you start taking it and each time you refill your prescription.
• After starting this drug, if you get an infection, any sign of an infection including a fever, cough, flu-like symptoms, or have any open sores on your body, call your healthcare provider right away. This drug should be stopped if you develop a serious infection.
• Before you begin treatment with this drug, talk with your healthcare provider about your vaccination history. Ask whether you should receive any vaccinations, including pneumonia vaccine and flu vaccine, before you begin this drug.