Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Intravenous immunoglobulin (IVIg) should be the initial therapy for most CIDP patients, dosed at 2 g/kg divided over 2-5 days, with approximately 80% of patients responding within 2-4 days. 1
First-Line Treatment Selection
The three established first-line therapies for CIDP are IVIg, corticosteroids, and plasma exchange, all with similar efficacy but different practical considerations 2, 3, 4:
IVIg (Preferred Initial Option)
- Standard dosing: 2 g/kg divided over 2-5 days 1
- Onset of action: 2-4 days, significantly faster than corticosteroids 1
- Response rate: approximately 80% initially 1
- Duration of effect: typically 3-4 weeks, requiring maintenance therapy 1
- Common side effects: headaches, fever, chills, fatigue 1
- Serious but rare complications: renal insufficiency, aseptic meningitis, thrombosis 1
- Best for: most patients, especially elderly and those with comorbidities (diabetes, obesity, hypertension) where corticosteroid side effects are concerning 4
Corticosteroids (Alternative First-Line)
Three regimens show equivalent efficacy with 60% response rate and 61% remission rate among responders 3:
- Daily oral prednisolone/prednisone: 1 mg/kg/day 3
- Pulsed oral dexamethasone 3
- Pulsed intravenous methylprednisolone 3
Key advantage: inexpensive and easily available, making it preferred for young, otherwise healthy patients requiring long-term therapy 4. The probability of achieving 5-year remission is 55% with no difference between the three corticosteroid regimens 3.
Important caveat: adverse events requiring treatment change occur in 8% of patients 3. Corticosteroids are less ideal in elderly patients and those with diabetes, obesity, or hypertension due to predictable side effects 4.
Plasma Exchange (Alternative First-Line)
- Proven efficacy equivalent to IVIg and corticosteroids 4, 5
- Best for: elderly patients and those with complicating medical illnesses where IVIg or corticosteroids pose higher risks 4
- Major limitations: transient effects, expensive, requires vascular access, only available in specialized centers, making long-term monotherapy problematic 4
Maintenance Therapy Strategy
After initial response to IVIg, effects typically last 3-4 weeks, necessitating maintenance therapy 1. For patients with treatment-related fluctuations (TRFs), repeating the full IVIg course is standard practice 1.
For long-term management, transition from IVIg or plasma exchange to prednisone is preferred in young, healthy patients due to cost and availability 4. IVIg is not ideal for long-term administration due to prohibitive costs 4.
Second-Line Therapies for Refractory Cases
When patients require repeated IVIg/plasma exchange and cannot tolerate prednisone, or require high-dose prednisone, add immunosuppressants 4:
- Azathioprine 4, 5
- Cyclosporin A 4, 5
- Cyclophosphamide 4, 5
- Mycophenolate mofetil 5
- Tacrolimus 5
- Rituximab (emerging therapy) 2, 5
These are typically used in combination with first-line therapies, not as monotherapy 4.
Critical Treatment Pitfalls
Avoid prolonged use of multiple immunosuppressive agents in refractory patients, as cumulative immunosuppression significantly increases morbidity and mortality risk beyond the acceptable risk of individual agents 6. Approximately 20% of CIDP patients become refractory to multiple immunosuppressive therapies and dependent on long-term intermittent plasma exchange, with substantial associated morbidity 6.
Early diagnosis and treatment yield better functional recovery by minimizing secondary axonal loss from primary demyelination 5. Most patients require prolonged therapy, making long-term side effects a critical consideration in treatment selection 5.