Hypogammaglobulinemia, secondary immunodeficiency disorders (SID)

(See separate entries for Kawasaki disease; for necrotizing fasciitis and toxic shock syndrome (TSS); and for transplant-related immunomodulation (solid organ transplant))

Recommended Indications in which IG can be used

Order Number: I2-SID

RecommendationsImmunoglobulin replacement is recommended for secondary prevention of recurrent, severe infection due to hypogammaglobulinemia (excluding paraprotein) related to other diseases or medical therapy in patients who have a history of infections. It is not recommended for routine replacement of IG as primary prophylaxis against infections in the setting of an isolated low IgG level without 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
The decision to use IG should be made in consultation with a physician with recognized expertise in immunodeficiency disorders. Hypogammaglobulinemia secondary to underlying disease or medical therapy (including HSCT) with all the following:
  • Serum IgG less than the lower limit of the reference range on two separate occasions
    AND
    At least one of the following:
    • One invasive or life-threatening infection (e.g., pneumonia, meningitis, sepsis) in the previous year.
    • Recurrent, severe infections.
    • Clinically active bronchiectasis confirmed by radiology; or
    • Assessment by a physician specializing in immunodeficiency indicating a significant antibody defect that would benefit from immunoglobulin replacement.
Dose/Frequency of AdministrationMaintenance: 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.

Disseminated enterovirus infection: One dose of 2g/kg adjusted body weight (IVIG or SCIG) divided over 2 to 5 days at any stage is permitted (in
addition to the maintenance dose).

Review Criteria
The following outcome measures should be recorded:
  • IgG level within 3 to 6 months; and
  • number of infections and hospital admissions for infection
Cessation of IG treatment may be possible depending on the status of the underlying disease.