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Cryopyrin Associated Periodic Syndromes

Updated : June 21, 2024





Background

Epidemiology

Anatomy

Pathophysiology

Etiology

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

rilonacept 

loading dose:

320

mg

Subcutaneous (SC)

Single dose


maintenance dose:160 mg subcutaneously administered once every week

note:
4.4 mg/kg subcutaneously administered once weekly
(not to exceed 320 mg)



canakinumab 

For individuals weighing over 40 kg, the recommended dosage is 150 mg administered subcutaneously every eight weeks
For individuals weighing between 15 to 40 kg, the initial dosage is 2 mg/kg of body weight, administered subcutaneously every eight weeks
The recommended dosage can be increased to 3 mg/kg If there is an inadequate response to the initial dose



anakinra 

Administer an initial dose of 1 to 2 mg/kg subcutaneously daily
Then it may raise up to 0.5 to 1 mg/kg
Dose should not be more than 8 mg/kg



 

rilonacept 

Age: 12-17 years
loading dose:

4.4

mg/kg

Subcutaneous (SC)


It is essential to ensure that the total dose does not exceed 320 mg

maintenance dose: 2.2 mg/kg via subcutaneous
do not exceed 160 mg or 2 mL per injection

Age: >18 years

loading dose: 320 mg subcutaneous once

maintenance dose: 160 mg subcutaneous injection once a week



canakinumab 

For Age ≥4 Years
For individuals weighing between 15-40 kg, administer 2 mg/kg subcutaneously every eight weeks
For individuals weighing 40 kg or more, administer 150 mg subcutaneously every eight weeks



anakinra 

Administer an initial dose of 1 to 2 mg/kg subcutaneously daily
Then it may raise up to 0.5 to 1 mg/kg
Dose should not be more than 8 mg/kg
Dosing Considerations
Measure white blood cells before starting, then every month up to 3 months and then quarterly up to one year



 

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References

Cryopyrin Associated Periodic Syndromes

Updated : June 21, 2024




rilonacept 

loading dose:

320

mg

Subcutaneous (SC)

Single dose


maintenance dose:160 mg subcutaneously administered once every week

note:
4.4 mg/kg subcutaneously administered once weekly
(not to exceed 320 mg)



canakinumab 

For individuals weighing over 40 kg, the recommended dosage is 150 mg administered subcutaneously every eight weeks
For individuals weighing between 15 to 40 kg, the initial dosage is 2 mg/kg of body weight, administered subcutaneously every eight weeks
The recommended dosage can be increased to 3 mg/kg If there is an inadequate response to the initial dose



anakinra 

Administer an initial dose of 1 to 2 mg/kg subcutaneously daily
Then it may raise up to 0.5 to 1 mg/kg
Dose should not be more than 8 mg/kg



rilonacept 

Age: 12-17 years
loading dose:

4.4

mg/kg

Subcutaneous (SC)


It is essential to ensure that the total dose does not exceed 320 mg

maintenance dose: 2.2 mg/kg via subcutaneous
do not exceed 160 mg or 2 mL per injection

Age: >18 years

loading dose: 320 mg subcutaneous once

maintenance dose: 160 mg subcutaneous injection once a week



canakinumab 

For Age ≥4 Years
For individuals weighing between 15-40 kg, administer 2 mg/kg subcutaneously every eight weeks
For individuals weighing 40 kg or more, administer 150 mg subcutaneously every eight weeks



anakinra 

Administer an initial dose of 1 to 2 mg/kg subcutaneously daily
Then it may raise up to 0.5 to 1 mg/kg
Dose should not be more than 8 mg/kg
Dosing Considerations
Measure white blood cells before starting, then every month up to 3 months and then quarterly up to one year