Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
First-line treatment for CIDP includes corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange, with early intervention being crucial to prevent permanent nerve damage. 1, 2
First-Line Treatment Options
Intravenous Immunoglobulin (IVIg)
- Standard initial therapy with proven efficacy
- Typical dosing: 2 g/kg divided over 2-5 days for induction
- Maintenance: 1 g/kg every 3 weeks, with adjustments based on clinical response
- Benefits: Rapid onset of action, generally well-tolerated
- Limitations: High cost, requires venous access, risk of infusion reactions
Subcutaneous Immunoglobulin (SCIg)
- Newer alternative to IVIg
- Advantages: Can be self-administered at home, more stable serum IgG levels, fewer systemic side effects
- Particularly useful for maintenance therapy after stabilization with IVIg 3
Corticosteroids
- Oral prednisone: 60-100 mg daily or alternate-day regimen with gradual taper
- Pulse intravenous methylprednisolone can be considered for severe cases
- Benefits: Low cost, oral administration
- Limitations: Significant side effects with long-term use (osteoporosis, diabetes, hypertension, weight gain)
Plasma Exchange
- Typically 2-3 exchanges per week for 2-3 weeks
- Useful for patients with acute deterioration or those who fail other therapies
- Limitations: Requires specialized equipment, venous access, and carries risk of hypotension and citrate toxicity
Treatment Algorithm
Initial Assessment:
- Confirm diagnosis using clinical, electrophysiological, and laboratory criteria
- Assess severity using validated disability scales
First-line Treatment Selection:
- For rapid improvement: Start with IVIg (2 g/kg over 2-5 days)
- For long-term management with fewer side effects: Consider corticosteroids
- For patients with contraindications to IVIg or steroids: Use plasma exchange
Response Evaluation:
- Assess clinical response after 2-4 weeks
- If improvement: Continue treatment and consider maintenance therapy
- If inadequate response: Switch to alternative first-line treatment
Maintenance Therapy:
- IVIg: Typically 1 g/kg every 3 weeks, adjusted based on response
- Corticosteroids: Gradual taper to lowest effective dose
- Consider transitioning from IVIg to SCIg for long-term management 3
For Refractory Cases:
- Consider immunosuppressants (azathioprine, mycophenolate mofetil, methotrexate)
- Rituximab may be considered in special cases 1
Treatment Optimization
- Personalize treatment schedule: Determine minimum effective dose and maximum interval between treatments to minimize side effects and cost
- Consider treatment holidays: Attempt periodic withdrawal of therapy in stable patients to assess ongoing need
- Monitor for treatment complications: Regular assessment for side effects of immunotherapy
Supportive Care
- Physical therapy to maintain strength and prevent contractures
- Pain management for neuropathic pain (gabapentin, pregabalin, duloxetine)
- Adaptive equipment for functional limitations
- Regular monitoring of respiratory function in patients with significant weakness
Special Considerations
- CIDP with diabetes: More challenging to diagnose; may require more aggressive treatment
- CIDP with axonal loss: May have incomplete recovery despite adequate immunotherapy
- Elderly patients: Higher risk of side effects from steroids; may prefer IVIg
Treatment Monitoring
- Regular clinical assessments using validated disability scales
- Electrophysiological studies to monitor improvement in demyelination
- Regular assessment for treatment side effects
Early diagnosis and prompt initiation of immunotherapy are critical to prevent irreversible axonal damage and disability in CIDP patients. The choice between first-line therapies should be based on patient factors, disease severity, and potential side effects.