From the Guidelines
No, IVIG (Intravenous Immunoglobulin) does not help all autoimmune conditions. IVIG is primarily effective for specific autoimmune disorders, including Kawasaki disease, as demonstrated by a study published in Circulation in 2017 1, which showed that IVIG reduces the prevalence of coronary artery abnormalities in patients with Kawasaki disease. The effectiveness of IVIG varies significantly between different autoimmune diseases, with some showing excellent response rates while others demonstrate minimal or no benefit. For example, in adult-onset Still's disease, IVIG has been used to treat flares and disease refractory to NSAIDs, with responses seen at doses ranging from 0.4 to 2 g/kg/day for 2–5 days and remission lasting for 2–53 months 1. However, for many autoimmune conditions, other treatments like corticosteroids, disease-modifying antirheumatic drugs, or targeted biologics remain the first-line options, with IVIG reserved for refractory cases or specific indications. Key considerations for the use of IVIG include:
- Dose: typically administered at doses ranging from 0.4-2 g/kg body weight
- Administration: often given over 2-5 consecutive days, with maintenance treatments scheduled every 3-4 weeks depending on the condition and response
- Mechanism of action: multiple mechanisms including blocking Fc receptors, neutralizing autoantibodies, inhibiting complement activation, and modulating cytokine production
- Indications: primarily effective for antibody-mediated autoimmune conditions, such as Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), immune thrombocytopenia (ITP), myasthenia gravis, and certain inflammatory myopathies.
From the Research
Efficacy of IVIG in Autoimmune Conditions
- IVIG has been shown to be effective in treating certain autoimmune neuromuscular diseases, including Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and multifocal motor neuropathy 2.
- It is also used to treat myasthenia gravis, particularly in patients with a suboptimal response or contraindications to prednisone or other immunosuppressive agents 2.
- IVIG has been demonstrated to be beneficial in placebo-controlled, double-blind, randomized studies in dermatomyositis and Lambert-Eaton syndrome 2.
- However, clinical trials in amyotrophic lateral sclerosis, inclusion body myositis, and anti-myelin-associated glycoprotein neuropathy have been negative 2.
Mechanisms of Action
- IVIG has multiple mechanisms of action, including the ability to neutralize pathogenic autoantibodies via anti-idiotypic antibodies within the IVIG preparation 3.
- It can also modulate the immune system by inducing IL-12 secretion, which activates natural killer cells, and inducing the expression of proapoptotic genes in cancer cells 3.
- IVIG can suppress pathogenic cytokines, inhibit complement binding, and prevent membranolytic attack complex (MAC) formation 4.
Off-Label Use
- IVIG is used off-label for a wide range of autoimmune diseases, including those that are not approved by the US Food and Drug Administration 5, 6.
- The use of IVIG in these conditions is based on clinical experience and published data, but the evidence may not be as strong as for approved indications 5, 6.
Safety and Adverse Effects
- IVIG is generally well-tolerated, with rare and mostly mild and transient side effects 3.
- However, patients prone to develop thrombotic events should be advised about the risk of IVIG therapy, especially at high doses 6.
- The high cost of IVIG therapy is a significant consideration, and clinicians should balance efficiency versus cost when deciding whether to treat certain conditions with IVIG 3.