Does intravenous immunoglobulin (IVIG) have a role in the treatment of Guillain‑Barré syndrome (GBS)?

IVIG Has a Definitive Role in Guillain-Barré Syndrome

Yes, intravenous immunoglobulin (IVIG) is the preferred first-line treatment for Guillain-Barré syndrome in patients who cannot walk independently, and should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1, 2

When to Initiate IVIG Treatment

IVIG therapy is strongly indicated for patients meeting any of the following criteria:

• Inability to walk independently (GBS disability score ≥ 3) 1
• Rapidly progressive limb weakness requiring immediate intervention 1
• Bulbar involvement including dysphagia or facial weakness 1
• Respiratory compromise indicated by:
  • Vital capacity < 20 mL/kg 1
  • Maximum inspiratory pressure < 30 cmH₂O 1
  • Maximum expiratory pressure < 40 cmH₂O 1
  • Inability to count to 15 on a single breath 1
  • Use of accessory respiratory muscles or breathlessness at rest 1

Approximately 20% of GBS patients will require mechanical ventilation, making early identification of respiratory indicators essential. 1

Standard IVIG Dosing Protocol

The recommended regimen is 0.4 g/kg/day intravenously for 5 consecutive days (total dose 2 g/kg). 1, 2, 3 This 5-day protocol is strongly preferred over accelerated 2-day regimens, which are associated with higher rates of treatment-related fluctuations. 1

Why IVIG is Preferred Over Plasma Exchange

While IVIG and plasma exchange are equally effective in hastening recovery and reducing long-term morbidity, IVIG is generally preferred as first-line therapy for several practical reasons: 1, 2

• Easier to administer without specialized equipment 1
• More widely available in most healthcare settings 1
• Higher treatment completion rates 1
• Fewer adverse effects compared to plasma exchange 1
• Better tolerability, especially in children and pregnant women 1

Plasma exchange may be considered when cost is a significant limiting factor, as it is substantially less expensive than IVIG in resource-limited settings. 1

Timing is Critical for Optimal Outcomes

Initiating IVIG within 2 weeks of symptom onset provides the greatest benefit for hastening recovery. 1, 2 Research demonstrates that treatment within the first 5 days reduces hospitalization length by approximately 11 days compared to later treatment. 4

The pharmacokinetics of IVIG vary considerably between patients, with those showing smaller increases in serum IgG levels (ΔIgG) after standard dosing experiencing slower recovery and worse outcomes at 6 months. 5 This suggests some patients may benefit from higher dosing or a second course, though this remains under investigation. 6

Special Population Considerations

Children

• IVIG is strongly preferred over plasma exchange due to better tolerability and fewer complications 1, 2
• Use the same 5-day regimen (0.4 g/kg/day) rather than accelerated protocols 1

Pregnant Women

• Neither IVIG nor plasma exchange is contraindicated during pregnancy 1
• IVIG is generally preferred because it requires fewer monitoring considerations 1, 2

Severe ICU-Level Disease

• Admit to a unit capable of rapid ICU transfer 1, 3
• Initiate IVIG immediately upon diagnosis 1
• For immune-checkpoint-inhibitor-related GBS, consider adding pulse-dose methylprednisolone (1 g daily for 5 days) to IVIG 1

Critical Treatment Caveats

Do NOT combine plasma exchange followed by IVIG sequentially—this approach offers no additional benefit, and plasma exchange performed after IVIG removes the administered immunoglobulin. 1

Corticosteroids alone are contraindicated in idiopathic GBS. Eight randomized controlled trials showed no benefit, and oral steroids were associated with worse outcomes. 1, 2 Corticosteroids are only appropriate in the specific context of immune checkpoint inhibitor-related GBS. 1

Variant-Specific Recommendations

Miller Fisher Syndrome

• Routine immunotherapy is generally not recommended because the disease course is usually mild with complete recovery within 6 months 1
• Close monitoring remains essential as some patients may develop limb weakness or respiratory failure 1

Bickerstaff Brainstem Encephalitis

• The severity justifies treatment with IVIG or plasma exchange 1

Evidence of Efficacy by Subtype

Recent research suggests IVIG efficacy may vary by GBS subtype. In acute inflammatory demyelinating polyradiculoneuropathy (AIDP), IVIG resulted in significantly fewer patients with poor recovery at 6 months (0.8% vs. 6.6%, p = 0.03). However, in acute motor axonal neuropathy (AMAN), IVIG therapy did not significantly alter outcomes compared to natural course. 7 Despite this finding, current guidelines do not differentiate treatment recommendations by subtype, and IVIG remains first-line therapy for all GBS patients meeting treatment criteria. 1, 2

Expected Outcomes

Approximately 80% of patients regain walking ability at 6 months after disease onset. 1, 2, 3 Mortality occurs in 3-10% of cases, most commonly from cardiovascular and respiratory complications. 1, 2, 3 Advanced age, severe disease at onset, and lack of ICU support when needed are risk factors for poor outcome. 1