Unifying Concepts

Intravenous Immune Globulin in Autoimmune and Inflammatory Diseases

Initially used for the treatment of immunodeficiencies, intravenous immunoglobulins (IVIg) have increasingly been used as immunomodulatory agent in autoimmune and inflammatory disorders. The mode of action of IVIg is enigmatic, probably involving Fc-dependent and/or F(ab’)2-dependent non-exclusive mechanisms of action. IVIg broadly interacts with the different components of the immune system: cytokines, complement, Fc receptors and several cell surface immunocompetent molecules. IVIg also has an impact on effector functions of immune cells. These mechanisms of action of IVIg reflect the importance of natural antibodies in the maintenance of immune homeostasis.

Diseases for Which Intravenous Immune Globulin Has Been Shown to Be Beneficial

FDA-approved indications

  • Primary immunodeficiency disease
  • Chronic lymphocytic leukemia
  • Pediatric HIV infection
  • Kawasaki’s disease
  • Allogeneic bone marrow transplantation
  • Chronic inflammatory demyelinating polyneuropathy
  • Kidney transplantation involving a recipient with a high antibody titer or an ABO-incompatible donor
  • Multifocal motor neuropathy

Additional approved indications with criteria
Neuromuscular disorders

  • Guillain–Barré syndrome
  • Relapsing–remitting multiple sclerosis
  • Myasthenia gravis
  • Refractory polymyositis
  • Polyradiculoneuropathy
  • Lambert–Eaton myasthenic syndrome
  • Opsoclonus–myoclonus
  • Birdshot retinopathy
  • Refractory dermatomyositis

Hematologic disorders

  • Autoimmune hemolytic anemia
  • Severe anemia associated with parvovirus B19
  • Autoimmune neutropenia
  • Neonatal alloimmune thrombocytopenia
  • HIV-associated thrombocytopenia
  • Graft-versus-host disease
  • Cytomegalovirus infection or interstitial pneumonia in patients undergoing bone marrow transplantation

Dermatologic disorders

  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • Bullous pemphigoid
  • Mucous-membrane (cicatricial) pemphigoid
  • Epidermolysis bullosa acquisita
  • Toxic epidermal necrolysis or Stevens–Johnson syndrome
  • Necrotizing fasciitis

* This is an abbreviated list of conditions approved under Medicare Part D or Aetna Clinical Policy Bulletin (2012). Criteria include medical certainty of diagnosis, medical necessity owing to the failure of usual treatments, contraindications to usual treatments, rapid progression or relapse, documentation of progress, and attempts to adjust drug dosages without improvement. FDA denotes Food and Drug Administration, and HIV human immunodeficiency virus.



  1. Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2012 Nov 22;367(21):2015-25. [Medline]
  2. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. [Medline]


Created Oct 16, 2013.

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