18. Intravenous Immune Globulin (IVIG)

Clinical Uses

IVIG is administered either to protect the recipient from infection or to suppress immune-mediated or inflammatory disease processes. While it is used off-label in nearly 100 conditions with probable or proven benefit, it is FDA approved for only eight: 

  • Immune thrombocytopenic purpura (ITP): generally chronic, treatment-refractory ITP or given to emergently increase a platelet count in a bleeding ITP patient.
  • Primary immunodeficiency.
  • Secondary immunodeficiency.
  • Pediatric HIV infection.
  • Kawasaki disease.
  • Prevention of GVHD in bone marrow transplant patients.
  • Incompatible solid organ transplants.
  • Chronic inflammatory demyelinating polyneuropathy (CIDP).
  • Common off-label uses include:
    • Hematologic: autoimmune hemolytic anemia, heparin-induced thrombocytopenia, autoimmune neutropenia, post-transfusion purpura, aplastic anemia, hypogammaglobulinemia associated with multiple myeloma.
    • Renal/vasculitis: membranous nephropathy, IgA nephropathy, lupus nephritis, GPA.
    • Neuromuscular: polymyositis, dermatomyositis, Guillain-Barré syndrome, myasthenia gravis.
    • Dermatologic: pemphigus vulgaris, toxic epidermal necrolysis and Stevens-Johnson syndrome.

Mechanisms of Action

Incompletely understood, but likely include the following:

  • Regulation and inhibition B-cells and T-cells.
  • Suppression of antibody production.
  • Modulation of complement.
  • Reduction of apoptosis.
  • Inhibition of cell-mediated cytotoxicity.
  • Inhibition of leukocyte adhesion.
  • Blockage of macrophage receptors.

Risks

  • During administration:
    • Flushing, tightness, back pain, nausea, chills, diaphoresis, headache, fever, hypotension.
    • Anaphylaxis (rare reaction in patients with high titers of anti-IgA antibodies).
  • Dose-related adverse effects:
    • Vascular: cerebral and coronary thromboses.
    • Infection: as with any blood product, small risk of viral hepatitis or HIV.
    • Neurologic: acute aseptic meningitis.
    • Hematologic: hemolysis (self-limited) and neutropenia (transient).
    • Renal: reversible renal impairment (can cause acute renal failure from osmotic nephropathy).
    • Electrolyte abnormalities: hyponatremia, pseudohyponatremia, decreased anion gap, false positive serologies.

 

Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol 2005;142:1-11.

Karafin MS, Shaz BH. Chapter 37: Human Immunoglobulin Preparations In: Shaz BH, Hillyer DC, Abrams SC, Roshal M eds. Transfusion Medicine and Hemostasis: Clinical and Laboratory Aspects, 2e. San Diego, CA: Elsevier; 2013.

Mouthon L, Lortholary O. Intravenous immunoglobulins in infectious diseases: where do we stand? Clin Microbiol Infect 2003;9:333-338.

Sacher RA; Intravenous immunoglobulin consensus statement. J Allergy Clin Immunol 2001;108:S139-146.