Intravenous Immunoglobulin: Comprehensive Guide, Uses & Risks

Authoritative facts about intravenous immunoglobulin (IVIG) from DermNet New Zealand dermatology and immunology experts.

What is intravenous immunoglobulin?

Intravenous immunoglobulin (IVIG) is a sterile preparation of concentrated antibodies (immunoglobulins) extracted from the plasma of thousands of healthy donors. These antibodies, primarily IgG, are proteins produced by the immune system to neutralise pathogens such as bacteria and viruses. IVIG serves as replacement therapy for patients with deficient or dysfunctional antibody production and modulates immune responses in autoimmune and inflammatory conditions.

The manufacturing process involves pooling plasma from screened donors, testing for infectious agents like HIV and hepatitis, fractionating to isolate immunoglobulins, purifying through cold ethanol precipitation and viral inactivation steps, and formulating into a 5 610% solution stabilised with sugars or amino acids. Products vary in IgA content and stabilisers, influencing suitability for specific patients.

Who needs IVIG therapy?

IVIG is indicated for primary immunodeficiencies (e.g., common variable immunodeficiency, X-linked agammaglobulinaemia), secondary immunodeficiencies from haematological malignancies, chemotherapy, or immunosuppressive drugs, and a range of autoimmune, inflammatory, and neurological disorders. In dermatology, it treats severe, refractory cases unresponsive to conventional therapies.

Immune deficiencies

• Primary antibody deficiencies where patients suffer recurrent sinopulmonary infections, bronchiectasis, or gastrointestinal issues due to low IgG levels.
• Secondary deficiencies post-bone marrow transplant, in multiple myeloma, or chronic lymphocytic leukaemia.

Autoimmune diseases

• Kawasaki disease to prevent coronary artery aneurysms.
• Immune thrombocytopenia (ITP) to raise platelet counts rapidly.
• Guillain-Barr e9 syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) for faster recovery.

Dermatological conditions

IVIG is a third- or fourth-line option for steroid-refractory or dependent cases:

• Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS): 48% improvement rate, reduces mortality by blocking Fas-mediated keratinocyte apoptosis.
• Pemphigus vulgaris/foliaceus: Effective in 70 690% as adjuvant or monotherapy.
• Bullous pemphigoid: Rapid control in severe cases.
• Mucous membrane pemphigoid: Especially ocular scarring variants.
• Epidermolysis bullosa acquisita (EBA), bullous systemic lupus erythematosus (BSLE).
• Pyoderma gangrenosum, autoimmune urticarias (chronic, solar, cold).
• Neutrophilic dermatoses: Erythema elevatum diutinum, Sweet syndrome.
• Atopic dermatitis: Severe paediatric/adult cases.
• Psoriasis: Erythrodermic, pustular, palmoplantar types.

Neurological disorders

• Multifocal motor neuropathy (MMN), myasthenia gravis, multiple sclerosis (MS), stiff person syndrome (SPS).

How does IVIG work?

IVIG exerts multiple immunomodulatory effects beyond antibody replacement:

• Neutralises pathogens and toxins via donor antibodies.
• Modulates immune cells: Inhibits B-cell activation, promotes regulatory T-cells, affects dendritic cells and macrophages.
• Blocks Fc receptors on phagocytes, reducing autoantibody clearance.
• Anti-idiotypic antibodies neutralise pathogenic autoantibodies.
• Suppresses pro-inflammatory cytokines (TNF-, IL-1) and complement activation.
• Inhibits adhesion molecules, reducing leucocyte migration to inflamed sites.

In dermatology, for SJS/TEN, IVIG saturates Fas receptors, preventing keratinocyte apoptosis. In pemphigus, it targets anti-desmoglein antibodies.

IVIG administration

IVIG is infused intravenously over 2 6 hours, typically 0.4 62 g/kg monthly, adjusted by condition and response. Premedication with paracetamol, diphenhydramine, or hydrocortisone mitigates reactions. Infusion rates start slow (e.g., 0.5 61 ml/kg/h) increasing if tolerated. Hospital or infusion centre administration is standard, though home infusion is possible for stable patients.

Side effects of IVIG

Most reactions are mild (headache, chills, myalgia, nausea) during infusion, managed by slowing rate or premedication. Serious effects (<5%) include aseptic meningitis, thromboembolism (stroke, MI, especially >2g/kg or risk factors), acute kidney injury (osmotic nephrosis from sucrose-stabilised products), and anaphylaxis (IgA-deficient patients with anti-IgA antibodies).

• Rate-related: Fever, rigors, hypotension 6 pause/slow infusion.
• Systemic: Thrombosis risk higher in elderly, dehydrated, or vascular disease patients.
• Renal: Avoid sucrose-containing IVIG in renal impairment.
• Neurological: Headache, aseptic meningitis (lumbar puncture confirmatory).

Monitoring during IVIG

Pre-infusion: Vital signs, IgA level (if history suggests deficiency), renal function, volume status. During: Continuous monitoring first 30 min, then hourly; stop if severe reaction. Post: Watch for delayed effects 24 672h. Long-term: IgG trough levels, infection frequency, platelet counts.

Outlook

IVIG significantly reduces infection rates in immunodeficiencies (from >5 to <1/year), accelerates recovery in GBS/ITP, and induces remission in refractory dermatoses (e.g., 75% pemphigus response). Quality of life improves markedly, though lifelong therapy may be needed. Costly (~$5000 610000/g) but cost-effective vs. hospitalisations.

Alternatives to IVIG

• Subcutaneous immunoglobulin (SCIG): Weekly home self-infusion, fewer systemic reactions.
• Immunosuppressants: Steroids, rituximab, cyclophosphamide for autoimmune diseases.
• Plasma exchange: Acute autoantibody removal.
• Biologics: Anti-CD20, IV belimumab.

Frequently asked questions

Is IVIG safe in pregnancy?

Yes, IVIG is considered safe and used for ITP, GBS, Kawasaki in pregnancy.

How quickly does IVIG work?

Hours to days in ITP/GBS; weeks in dermatological/autoimmune conditions.

Can IVIG cause infections?

No, donor screening and viral inactivation ensure safety.

What if I have renal disease?

Use maltose- or glycine-stabilised products; monitor closely.

Is home IVIG possible?

Yes, for stable primary immunodeficiency patients after training.

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Medha Deb is an editor with a master's degree in Applied Linguistics from the University of Hyderabad. She believes that her qualification has helped her develop a deep understanding of language and its application in various contexts.

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