Do we start Intravenous Immunoglobulin (IVIG) in Guillain-Barré Syndrome (GBS)?

IVIG Treatment in Guillain-Barré Syndrome

Yes, start IVIG (0.4 g/kg body weight daily for 5 days) in patients with GBS who are unable to walk unaided, as it is the first-line treatment of choice and equally effective as plasma exchange while being easier to administer. 1, 2

When to Initiate IVIG

Start IVIG immediately in any GBS patient who cannot walk independently (disability grade ≥3). 1, 2 Treatment should begin as early as possible in the disease course, ideally within 2 weeks of symptom onset, to maximize effectiveness. 2, 3

For patients with mild disease (still able to walk unaided):

• Close monitoring is essential, but treatment is generally not required unless progression occurs 1
• Reassess frequently for signs of deterioration

For severe or rapidly progressive disease (Grade 3-4):

• Admit to inpatient unit with ICU capability 1
• Start IVIG 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 2
• Monitor respiratory function continuously 1

Why IVIG Over Plasma Exchange

IVIG is preferred as first-line therapy because: 1, 2

• Easier to administer in most clinical settings
• More widely available than plasma exchange
• Higher completion rates (patients are significantly more likely to complete IVIG than plasma exchange) 3
• Comparable efficacy to plasma exchange for severe disease 1, 3
• Similar adverse event profile 1, 3

The evidence shows no significant difference in disability improvement at 4 weeks between IVIG and plasma exchange (mean difference 0.02 grade, 95% CI -0.20 to 0.25), confirming equivalent efficacy. 3

Critical Respiratory Monitoring

Use the "20/30/40 rule" to assess respiratory failure risk: 2, 4

• Vital capacity <20 ml/kg
• Maximum inspiratory pressure <30 cmH₂O
• Maximum expiratory pressure <40 cmH₂O

Additional warning signs requiring immediate ICU transfer: 1

• Breathlessness at rest or during talking
• Inability to count to 15 in a single breath
• Use of accessory respiratory muscles
• Single breath count ≤19 predicts need for mechanical ventilation 4

Up to 22% of GBS patients require mechanical ventilation within the first week, making early identification crucial. 1

Special Populations

Children: IVIG is preferred over plasma exchange due to better tolerability and fewer complications. 2 Low-quality evidence suggests IVIG hastens recovery compared to supportive care alone in pediatric GBS. 3

Pregnant women: Both IVIG and plasma exchange are not contraindicated, but IVIG is preferred due to fewer monitoring requirements. 1, 2

AMAN subtype (pure motor): Recent research suggests IVIG may not alter outcomes in acute motor axonal neuropathy (AMAN) compared to natural course, though it remains beneficial in AIDP variants. 5 However, current guidelines still recommend treating all GBS subtypes with IVIG. 1, 4

What NOT to Do

Do not use corticosteroids alone - eight randomized controlled trials showed no benefit, and oral corticosteroids may worsen outcomes. 1, 2

Do not combine plasma exchange followed by IVIG - this provides no additional benefit over either treatment alone. 1, 2

Avoid these medications that worsen neuromuscular function: 2

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

Managing Inadequate Response

If no improvement occurs within 4 weeks: 1

• This affects approximately 40% of patients and doesn't necessarily indicate treatment failure
• Disease progression might have been worse without therapy
• Evidence for repeating treatment or switching therapies is currently lacking

Treatment-related fluctuations (TRFs): 1, 4

• Occur in 6-10% of patients within 2 months of initial improvement
• Defined as disease progression after initial treatment-induced stabilization
• Repeating the full 5-day IVIG course is common practice, though evidence is limited
• Distinguish from patients who never responded initially

Pharmacokinetic considerations: 6

• Patients show large variation in serum IgG increase after standard IVIG dosing
• Those with low serum IgG increase (ΔIgG) 2 weeks post-treatment have significantly slower recovery
• This suggests some patients may benefit from higher dosing or a second course, though this requires further investigation 7

Expected Outcomes

Prognosis with IVIG treatment: 1, 4

• 80% of patients regain independent walking ability at 6 months
• Mortality occurs in 3-10% of cases, primarily from cardiovascular and respiratory complications
• Risk factors for poor outcome include advanced age and severe disease at onset
• Recovery can continue beyond 5 years after disease onset