Usual Adult Dose for Familial Cold Autoinflammatory Syndrome

15 kg less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks
Greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS)

Usual Adult Dose for Muckle Wells Syndrome

15 kg less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks
Greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS)

Usual Adult Dose for Cryopyrin-Associated Periodic Syndrome

15 kg less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks
Greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS)

Usual Adult Dose for Familial Mediterranean Fever

Up to 40 kg: 2 mg/kg subcutaneously every 4 weeks; dose can be increased to 4 mg/kg subcutaneously every 4 weeks if response is not adequate
Greater than 40 kg: 150 mg subcutaneously every 4 weeks; dose can be increased to 300 mg subcutaneously every 4 weeks if response is not adequate

Uses: For autoinflammatory periodic fever syndromes including periodic tumor necrosis factor receptor associated periodic syndrome (TRAPS), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (MKD), and familial Mediterranean fever (FMF)

Usual Adult Dose for Hyperimmunoglobulin D Periodic Fever Syndrome

Up to 40 kg: 2 mg/kg subcutaneously every 4 weeks; dose can be increased to 4 mg/kg subcutaneously every 4 weeks if response is not adequate
Greater than 40 kg: 150 mg subcutaneously every 4 weeks; dose can be increased to 300 mg subcutaneously every 4 weeks if response is not adequate

Uses: For autoinflammatory periodic fever syndromes including periodic tumor necrosis factor receptor associated periodic syndrome (TRAPS), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (MKD), and familial Mediterranean fever (FMF)

Usual Adult Dose for Tumor Necrosis Factor Receptor Associated Periodic Fever Syndrome

Up to 40 kg: 2 mg/kg subcutaneously every 4 weeks; dose can be increased to 4 mg/kg subcutaneously every 4 weeks if response is not adequate
Greater than 40 kg: 150 mg subcutaneously every 4 weeks; dose can be increased to 300 mg subcutaneously every 4 weeks if response is not adequate

Uses: For autoinflammatory periodic fever syndromes including periodic tumor necrosis factor receptor associated periodic syndrome (TRAPS), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (MKD), and familial Mediterranean fever (FMF)

Usual Adult Dose for Still's Disease

7.5 kg or greater: 4 mg/kg (maximum 300 mg) subcutaneously every 4 weeks

Use: Still's Disease, including Adult-Onset Still's Disease (AOSD)

Usual Pediatric Dose for Familial Cold Autoinflammatory Syndrome

4 years and older:

• Weight 15 kg to less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks; for children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg subcutaneously every 8 weeks
• Weight greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS) in children 4 years and older

Usual Pediatric Dose for Muckle Wells Syndrome

4 years and older:

• Weight 15 kg to less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks; for children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg subcutaneously every 8 weeks
• Weight greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS) in children 4 years and older

Usual Pediatric Dose for Cryopyrin-Associated Periodic Syndrome

4 years and older:

• Weight 15 kg to less than or equal to 40 kg: 2 mg/kg subcutaneously every 8 weeks; for children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg subcutaneously every 8 weeks
• Weight greater than 40 kg: 150 mg subcutaneously every 8 weeks

Use: Cryopyrin-associated periodic syndromes (CAPS) including familial cold autoinflammatory syndrome (FCAS) and muckle-wells syndrome (MWS) in children 4 years and older

Usual Pediatric Dose for Familial Mediterranean Fever

2 years and older:

• Weight 40 kg or less: 2 mg/kg subcutaneously every 4 weeks; can increase to 4 mg/kg subcutaneously every 4 weeks if response is inadequate
• Weight greater than 40 kg: 150 mg subcutaneously every 4 weeks; can increase to 300 mg subcutaneously every 4 weeks if response is inadequate

Use: For the treatment of autoinflammatory periodic fever syndromes including TRAPS, HIDS/MKD, and FMF in pediatric patients 2 years and older

Usual Pediatric Dose for Hyperimmunoglobulin D Periodic Fever Syndrome

2 years and older:

• Weight 40 kg or less: 2 mg/kg subcutaneously every 4 weeks; can increase to 4 mg/kg subcutaneously every 4 weeks if response is inadequate
• Weight greater than 40 kg: 150 mg subcutaneously every 4 weeks; can increase to 300 mg subcutaneously every 4 weeks if response is inadequate

Use: For the treatment of autoinflammatory periodic fever syndromes including TRAPS, HIDS/MKD, and FMF in pediatric patients 2 years and older

Usual Pediatric Dose for Tumor Necrosis Factor Receptor Associated Periodic Fever Syndrome

2 years and older:

• Weight 40 kg or less: 2 mg/kg subcutaneously every 4 weeks; can increase to 4 mg/kg subcutaneously every 4 weeks if response is inadequate
• Weight greater than 40 kg: 150 mg subcutaneously every 4 weeks; can increase to 300 mg subcutaneously every 4 weeks if response is inadequate

Use: For the treatment of autoinflammatory periodic fever syndromes including TRAPS, HIDS/MKD, and FMF in pediatric patients 2 years and older

Usual Pediatric Dose for Juvenile Idiopathic Arthritis

2 years and older:
7.5 kg or greater: 4 mg/kg (maximum 300 mg) subcutaneously every 4 weeks

Use: For the treatment of active systemic juvenile idiopathic arthritis (SJIA) in patients 2 years and older and 7.5 kg or more

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

CONTRAINDICATIONS:

• Hypersensitivity to the active component or any of the ingredients

Safety and efficacy have not been established in patients younger than 4 years with CAPS.
Safety and efficacy have not been established in patients younger than 2 years with TRAPS, HIDS/MKD, FMF.
Safety and efficacy have not been established in patients younger than 2 years or weighing less than 7.5 kg with SJIA.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Avoid injection into scar tissue.
• Vials are intended for single use in 1 patient; discard any unused portion.

Storage requirements:

• The unopened vial should be refrigerated 2C to 8C (36F to 46 F). Do not freeze.
• Store in the original carton to protect from light.
• Do not use beyond the date stamped on the label.
• This drug does not contain preservatives.

General:

• Do not use beyond the date stamped on the label.
• This drug does not contain preservatives.
• Discard any unused portion or waste material in accordance with local requirements.