What is the initial treatment for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Initial Treatment for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

The initial treatment for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) should be intravenous immunoglobulin (IVIg), corticosteroids, or plasma exchange, with IVIg often preferred as first-line therapy due to its rapid onset of action and favorable side effect profile compared to long-term corticosteroid use. 1, 2

First-Line Treatment Options

Intravenous Immunoglobulin (IVIg)

• Recommended as initial therapy for most CIDP patients due to rapid onset of action (typically within 2-4 days) 3
• Standard dosing: 2 g/kg divided over 2-5 days 3
• Approximately 80% of patients respond initially 3
• Effects typically last 3-4 weeks, requiring maintenance therapy 3
• Common side effects include headaches, fever, chills, and fatigue 3
• Rare but serious side effects include renal insufficiency, aseptic meningitis, and thrombosis 3

Corticosteroids

• Effective alternative first-line therapy with approximately 60% response rate 4
• Three main regimens with similar efficacy:
  • Daily oral prednisone/prednisolone (0.5-1 mg/kg/day) 4
  • Pulsed oral dexamethasone 4
  • Pulsed intravenous methylprednisolone 4
• Slower onset of action compared to IVIg 1
• Caution: Patients with pure motor CIDP may deteriorate with corticosteroid treatment 1
• Side effects include weight gain, hypertension, diabetes, osteoporosis, and mood changes 5

Plasma Exchange

• Effective alternative first-line therapy for patients who cannot tolerate or do not respond to IVIg or corticosteroids 1
• Typically requires specialized centers for administration 5
• Effects are usually transient, lasting 3-4 weeks 6

Treatment Algorithm

1. Initial assessment:

   • Confirm CIDP diagnosis based on clinical presentation, electrophysiological studies, and CSF analysis 2
   • Evaluate disease severity and functional impairment 5
   • Check for contraindications to specific therapies 1

2. First-line therapy selection:

   • For most patients: Start with IVIg at 2 g/kg divided over 2-5 days 3, 2
   • For patients with contraindications to IVIg (renal insufficiency, history of thrombosis, IgA deficiency): Consider corticosteroids or plasma exchange 3, 5
   • For pure motor CIDP: Avoid corticosteroids and prefer IVIg 1

3. Response evaluation:

   • Assess clinical response after 2-4 weeks 5
   • If inadequate response to initial therapy, switch to an alternative first-line treatment 1
   • If no response to any first-line therapy, consider combination therapy or immunosuppressive agents 2

Maintenance Therapy

• For IVIg responders: Maintenance infusions typically at 1 g/kg every 3-4 weeks, with dose and interval individualized based on response 3, 5
• For corticosteroid responders: Gradually taper dose after achieving remission 4
• Approximately 61% of corticosteroid responders achieve remission during follow-up 4
• The probability of 5-year remission for corticosteroid responders is approximately 55% 4

Special Considerations

• Check for monoclonal proteins or signs of malignancy in patients who become unresponsive to therapy 1
• For patients with treatment-related fluctuations (TRFs), repeating the full course of IVIg is common practice 3
• Distinguish between true CIDP and acute-onset CIDP (which typically presents with three or more TRFs and/or clinical deterioration ≥8 weeks after disease onset) 3

Refractory Cases

• For patients who fail to respond to first-line therapies, consider:
  • Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, methotrexate, mycophenolate mofetil) 1, 6
  • Rituximab (anti-CD20 monoclonal antibody) 2, 6
  • Combination therapy 5
• Evidence for these second-line treatments is less robust than for first-line therapies 1