Treatment of Immunological Disorders

Introduction to Immunoglobulin Therapy

The American Academy of Neurology recommends IVIG as a first-line therapy for Acute Motor-Sensory Axonal Neuropathy (AMSAN), administered at 1-2 g/kg divided over 2-5 consecutive days to prevent axonal degeneration and improve clinical outcomes 1
The American Academy of Allergy, Asthma, and Immunology recommends IVIG therapy for patients with primary immunodeficiency disorders, with a strength of evidence rated as high 2

The recommended dose of IVIG for AMSAN treatment is 1-2 g/kg of ideal body weight, usually divided over 2 consecutive days (1 g/kg each day) 1
The recommended dosage for primary humoral immunodeficiency disorders is 400 to 600 mg/kg every 3 to 4 weeks, with adjustments based on clinical condition and response, as suggested by the Immune Deficiency Foundation 3
For Kawasaki Disease, the recommended dose is 2 g/kg as a single infusion over 10-12 hours with aspirin, as recommended by the American Heart Association (AHA) 4
For Immune Thrombocytopenic Purpura, the recommended dose is 0.4 g/kg/day for 5 days or 1 g/kg/day for 1-2 days, as suggested by the American Society of Hematology (ASH) 5
For Autoimmune/Inflammatory Conditions, the typical dose is 2 g/kg divided over several days, given at monthly intervals, as recommended by the National Institute of Neurological Disorders and Stroke (NINDS) 6

Check serum IgA levels to prevent severe anaphylactic reactions in IgA-deficient patients, and consider using IgA-depleted IVIG preparations in patients with IgA deficiency 1
Assess renal function, as IVIG can cause acute kidney injury in susceptible patients 1
Regular monitoring of trough IgG levels and tracking the frequency and severity of infections is necessary for patients receiving IVIG therapy, as recommended by the Immune Deficiency Foundation 3
Patients receiving regular IVIG for immunodeficiency should have trough IgG levels monitored, and renal function, complete blood count, and signs of thrombosis should be monitored in patients receiving high-dose therapy, as suggested by the American Academy of Allergy, Asthma, and Immunology (AAAAI) 5

If IVIG is unavailable or contraindicated, consider plasma exchange (plasmapheresis) as an alternative first-line therapy with similar efficacy 1
Subcutaneous immunoglobulin (SCIG) could be considered as an alternative administration route that provides stable serum IgG levels with fewer systemic adverse events 2
Ensure correct diagnosis of AMSAN, as chronic inflammatory demyelinating polyneuropathy (CIDP) requires different treatment protocols and maintenance therapy 1
Live vaccines (measles, mumps, varicella) should be deferred for 11 months after receiving high-dose IVIG, as recommended by the Centers for Disease Control and Prevention (CDC) and the American Academy of Pediatrics 4, 7

Common adverse effects include headache, fever, chills, fatigue, nausea, and myalgia, as reported by the FDA 5, 6
Less common adverse effects include aseptic meningitis and hemolytic anemia (especially in individuals with AB blood type), as described by the National Institute of Neurological Disorders and Stroke (NINDS) 4
Rare but serious adverse effects include thrombosis, renal failure, anaphylaxis (particularly in IgA-deficient patients), and intravascular hemolysis, as warned by the FDA 6

IVIG is used to treat refractory Dermatomyositis, severe or refractory Pemphigus Vulgaris, and certain immunotherapy-related toxicities, as recommended by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) 6, 8, 9
Immunoglobulin is recommended for post-exposure prophylaxis of hepatitis A, with a dose of 0.02 mL/kg IM within 2 weeks after exposure, and for post-exposure prophylaxis of measles, with a dose of 0.25 mL/kg IM within 6 days after exposure, as suggested by the Centers for Disease Control and Prevention 10
The Centers for Disease Control and Prevention recommends following the annual vaccination schedule for patients with HIV 11
The Infectious Diseases Society of America recommends that inactivated vaccines not be administered routinely during immunoglobulin therapy for patients with major antibody deficiencies 12