Intravenous Immunoglobulin

OVERVIEW

Intravenous immunoglobulin (IVIG)

• initially developed for replacement therapy for patients with humoral immune deficiency
• immunomodulatory agent
• similar indications in autoimmune disease as for plasma exchange
• blood product prepared from the serum of between 1000 and 15,000 donors per batch

DOSE

•  ‘replacement dose’ of 200–400 mg/kg body weight, given approximately 3-weekly for antibody deficiencies
•  ‘high dose’ IVIG (hdIVIG) at 2 g/kg/month as an immunomodulatory agent

MECHANISM

• modulates T and B cells, macrophages -> interfering with antibody production and degradation
• modulates complement and cytokine networks
• regulation of cell growth

Four separate components:

• actions of variable regions F(ab′)2
• actions of Fc region on a range of Fc receptors (FcR)
• actions mediated by complement binding within the Fc fragment
• immunomodulatory substances other than antibody in the IVIG preparations (e.g. cytokines)

INDICATIONS

Haematological

• idiopathic thrombocytopenic purpura (ITP)
• post plasma exchange course completion
• antiphospholipid syndrome (APLS)
• chronic graft versus host disease (hypogammaglobulinaemia)

Infections

• toxic shock syndromes (staphyloccocal and streptococcal)

Vasculidities

• Kawasaki disease
• Granulomatosis with polyangiitis (GPA) (Wegener granulomatosis)
• Microscopic polyangiitis

Dermatological

• pemphigus vulgaris
• bullous pemphigoid
• SJS/TEN (controversial)

Neuromuscular

• GBS (AIDP)
• dermatomyositis
• myasthenia gravis
• chronic inflammatory demyelinating peripheral neuropathy (CIDP)
• Eaton-Lambert syndrome
• Stiff Person Syndrome
• Multiple sclerosis

Renal transplant rejection (controversial)

References and Links

• Momin SB. Review of intravenous immunoglobulin in the treatment of stevens-johnson syndrome and toxic epidermal necrolysis. J Clin Aesthet Dermatol. 2009 Feb;2(2):51-8. PMC2958184.
• Hartung HP, Mouthon L, Ahmed R, Jordan S, Laupland KB, Jolles S. Clinical applications of intravenous immunoglobulins (IVIg)–beyond immunodeficiencies and neurology. Clin Exp Immunol. 2009 Dec;158 Suppl 1:23-33. PMC2801038.
• Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. PMC1809480.
• Shankar-Hari M, Spencer J, Sewell WA, Rowan KM, Singer M. Bench-to-bedsidereview: Immunoglobulin therapy for sepsis – biological plausibility from a critical care perspective. Crit Care. 2012 Dec 12;16(2):206. doi:10.1186/cc10597. PMC3584720.