What is the recommended dosing of intravenous immunoglobulin (IVIG) for the treatment of Guillain-Barré Syndrome (GBS)?

IVIG Dosing in Guillain-Barré Syndrome

The recommended dosing of intravenous immunoglobulin (IVIG) for Guillain-Barré Syndrome (GBS) is 0.4 g/kg body weight daily for 5 consecutive days, for a total dose of 2 g/kg. 1, 2

First-Line Treatment Recommendations

• IVIG at 0.4 g/kg/day for 5 days (total 2 g/kg) is the standard first-line treatment for GBS patients who are unable to walk independently 1
• IVIG is generally preferred over plasma exchange due to:
  • Easier administration 1
  • Greater availability in most centers 1
  • Higher completion rates 1
  • Better tolerability, especially in children and pregnant women 3

Timing of Treatment

• Treatment should be initiated as early as possible in the disease course to maximize effectiveness 1
• Delaying treatment beyond 2 weeks after symptom onset may reduce efficacy 2

Special Patient Populations

Children

• IVIG is the preferred treatment for children with GBS 3
• While some pediatric centers administer the total dose over 2 days instead of 5, this approach has been associated with higher rates of treatment-related fluctuations (5/23 children with 2-day regimen vs. 0/23 with 5-day regimen) 3
• The standard adult regimen of 0.4 g/kg/day for 5 days is recommended for children 3

Pregnant Women

• IVIG is generally preferred over plasma exchange in pregnant women due to fewer monitoring requirements 3
• The standard dosing of 0.4 g/kg/day for 5 days is appropriate for pregnant women 3

GBS Variants

• For Miller Fisher Syndrome (MFS), treatment is generally not recommended due to the typically mild course with complete recovery within 6 months 3
• For Bickerstaff's brainstem encephalitis (BBE), IVIG treatment at standard doses is justified despite limited evidence 3
• For immune checkpoint inhibitor-related GBS, the standard IVIG dosing is recommended, potentially with concurrent corticosteroids 3

Monitoring Response and Treatment Failure

• Approximately 40% of patients do not improve within 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1
• A second course of IVIG is NOT recommended for patients with poor prognosis, as research shows no benefit and increased risk of serious adverse events, particularly thromboembolic events 4
• Regular monitoring of respiratory function is essential using:
  • Vital capacity measurements 3
  • Maximum inspiratory and expiratory pressures 3
  • The "20/30/40 rule" to assess respiratory failure risk 1

Common Pitfalls and Caveats

• Corticosteroids alone are not recommended for GBS treatment and may even have negative effects on outcomes 1
• Pharmacokinetics of IVIG can vary between patients, with some showing lower increases in serum IgG levels after standard dosing, which may be associated with poorer outcomes 5
• Avoid medications that can worsen neuromuscular function during IVIG treatment, including β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 2
• Up to two-thirds of deaths in GBS occur during the recovery phase, so continued vigilance is necessary even after apparent improvement 3

Outcome Measures

• Approximately 80% of patients regain walking ability within 6 months after disease onset 1
• Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 2
• Risk factors for poor outcomes include advanced age and severe disease at onset 1