Primary immunodeficiency (PID) disorders
Including by not limited to:
- common variable immunodeficiency (CVID) and associated disorders
- specific antibody deficiency
- IgG subclass deficiency
- combined immunodeficiency
Recommended Indications in which IG can be used
Order Number: I1-PID
| Recommendations | Immunoglobulin replacement is recommended for preventing infection. Note: This includes bacterial infections as well as select viral, protozoal, and fungal infections, as directed by a physician with recognized expertise in immunodeficiency disorders. Qualifying Criteria PID diagnosis must be established by a physician with recognized expertise in immunodeficiency disorders. Functional criteria are required to establish diagnosis for the following specific disorders:
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| Dose/Frequency of Administration | Maintenance: 0.4 to 0.6 g/kg adjusted body weight IVIG every 4 weeks or SCIG 0.1 to 0.15 g/kg adjusted body weight weekly, modified to achieve an IgG trough level of at least the lower limit of the age-specific serum IgG reference range, or as needed to achieve clinical effectiveness. Loading: One additional dose of 0.4 g/kg adjusted body weight may be given in the first month of therapy if the serum IgG level is markedly reduced. Chronic suppurative lung disease: 0.4 to 0.8 g/kg adjusted body weight IVIG or equivalent SCIG dose may be given if chronic suppurative lung disease is not adequately controlled at an IgG trough level at the lower limit of the age-specific serum IgG reference range. Specific antibody deficiency and IgG subclass deficiency: IVIG should be titrated based on clinical outcome alone as measurement of IgG trough levels is unhelpful in these conditions. Review Criteria The following outcome measures should be recorded:
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