What are the indications for intravenous immunoglobulin (IVIG) or plasma exchange (PLEX) in Guillain‑Barré syndrome?

Indications for IVIG or Plasma Exchange in Guillain-Barré Syndrome

Both IVIG (0.4 g/kg/day for 5 days) and plasma exchange (200-250 ml plasma/kg over 5 sessions) are equally effective first-line treatments for GBS patients who cannot walk unaided, and treatment should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1

Primary Treatment Indications

Treat any GBS patient with:

• Inability to walk independently (GBS disability score ≥3) 1, 2, 3
• Rapidly progressive weakness 1, 3
• Any dysphagia or bulbar weakness 1, 3
• Facial weakness 1, 3
• Respiratory muscle weakness 1

The Nature Reviews Neurology guidelines emphasize that treatment is indicated for patients requiring aid for walking, as both IVIG and plasma exchange have been proven equally effective in hastening recovery and reducing long-term morbidity in this population. 1

Critical Respiratory Indicators Requiring Immediate Treatment

Initiate treatment immediately if any of the following are present:

• Vital capacity <20 ml/kg 1, 3
• Maximum inspiratory pressure <30 cmH₂O 1, 3
• Maximum expiratory pressure <40 cmH₂O 1, 3
• Inability to count to 15 in a single breath 1
• Use of accessory respiratory muscles 1
• Breathlessness at rest or during talking 1

Approximately 20% of GBS patients require mechanical ventilation, making early identification and treatment of those at risk critical for preventing mortality. 1, 2, 3

Choosing Between IVIG and Plasma Exchange

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

• Easier to administer
• More widely available
• Higher treatment completion rates
• Fewer adverse effects and complications
• Does not require specialized equipment or expertise

Plasma exchange may be preferred when: 1, 3

• Cost is a major limiting factor (significantly less expensive than IVIG in resource-limited settings)
• Patient fails to respond to IVIG
• IVIG is unavailable or contraindicated

The 1997 Lancet trial definitively established that PE and IVIG have equivalent efficacy, with mean improvement of 0.9 disability grades for PE versus 0.8 for IVIG at 4 weeks, a difference so small that clinical equivalence was confirmed. 4

Treatment Timing

Optimal treatment window: 3

• Within 7 days of symptom onset (most beneficial)
• Up to 14 days (standard recommendation for trial enrollment)
• Up to 30 days (still beneficial, though less optimal)

Treatment initiated within 2 weeks of symptom onset provides the greatest benefit for hastening recovery. 1, 2, 3

Standard Dosing Regimens

IVIG: 1, 2

• 0.4 g/kg/day intravenously for 5 consecutive days
• Total cumulative dose: 2 g/kg

Plasma Exchange: 1

• 200-250 ml plasma/kg body weight
• Divided into 5 sessions over 8-13 days

Special Population Considerations

Children: 1, 2, 5

• IVIG is strongly preferred over plasma exchange due to better tolerability and fewer complications
• Use the same 5-day regimen (0.4 g/kg/day) as adults
• Avoid 2-day accelerated regimens, as they are associated with higher treatment-related fluctuation rates

Pregnant women: 1, 2, 5

• Neither IVIG nor plasma exchange is contraindicated during pregnancy
• IVIG is generally preferred due to fewer monitoring considerations and precautions required

ICU-level patients with severe disease (G3-4): 1

• Admit to inpatient unit with capability for rapid ICU transfer
• Initiate IVIG or plasmapheresis immediately
• Consider pulse-dose methylprednisolone (1 g daily for 5 days) in addition to IVIG or plasma exchange for immune checkpoint inhibitor-related GBS, though corticosteroids alone are not recommended for idiopathic GBS

Important Treatment Caveats

Do NOT combine plasma exchange followed by IVIG - this sequential approach is no more effective than either treatment alone and plasma exchange immediately after IVIG will remove the immunoglobulin. 1, 3 The 1997 Lancet trial showed mean improvement of 1.1 grades with combination therapy versus 0.9 for PE alone and 0.8 for IVIG alone, differences that were not statistically significant. 4

Corticosteroids alone are contraindicated - eight randomized controlled trials showed no benefit, and oral corticosteroids were shown to have negative effects on outcome. 1

Treatment-related fluctuations occur in 6-10% of patients within 2 months of initial improvement, and repeating the full course of the original treatment is common practice. 3

Variants Requiring Modified Approach

Miller Fisher Syndrome (pure MFS): 1

• Treatment generally not recommended due to relatively mild disease course
• Most recover completely without treatment within 6 months
• Monitor closely as a subgroup can develop limb weakness, bulbar palsy, or respiratory failure

Bickerstaff Brainstem Encephalitis (BBE): 1

• Severity justifies treatment with IVIG or plasma exchange
• Evidence for efficacy is limited but expert consensus supports treatment

Monitoring During Treatment

Essential monitoring includes: 1, 2, 3, 5

• Frequent pulmonary function testing (vital capacity, maximum inspiratory/expiratory pressures)
• Serial neurologic examinations using Medical Research Council grading scale
• GBS disability scale assessment
• Electrocardiography and autonomic function monitoring (heart rate, blood pressure, bowel/bladder function)
• Assessment for swallowing and coughing difficulties

The Erasmus GBS Respiratory Insufficiency Score (EGRIS) can calculate the probability (1-90%) that a patient will require ventilation within 1 week, helping identify high-risk patients requiring more intensive monitoring. 1, 2