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 either intravenous immunoglobulin (IVIG), corticosteroids, or plasma exchange, with IVIG often preferred as first-line therapy due to its favorable efficacy and side effect profile. 1, 2

First-Line Treatment Options

Intravenous Immunoglobulin (IVIG)

• IVIG is considered a first-line therapy for CIDP induction treatment 1
• Offers rapid onset of action and is particularly beneficial for patients who need quick improvement 2
• Now available in subcutaneous formulation (SCIG), which can increase patient independence and improve tolerability 3

Corticosteroids

• Effective as both induction and maintenance therapy for CIDP 1
• Three main corticosteroid regimens are used with similar efficacy:
  • Daily oral prednisolone/prednisone
  • Pulsed oral dexamethasone
  • Pulsed intravenous methylprednisolone 4
• Overall response rate to corticosteroids is approximately 60% of patients 4
• Among responders, about 61% achieve remission with a 55% probability of 5-year remission 4
• Side effects are a significant concern with long-term use, including moon facies, sleeplessness, and other serious adverse effects 5

Plasma Exchange

• Effective as an induction treatment for CIDP 1
• Typically reserved for patients with severe disease or those who don't respond to IVIG or corticosteroids 2

Treatment Algorithm

1. Initial Assessment:

   • Confirm diagnosis using internationally accepted criteria for CIDP 5
   • Evaluate disease severity, comorbidities, and contraindications to specific therapies 2

2. First-Line Treatment Selection:

   • For patients requiring rapid improvement: IVIG (2g/kg divided over 2-5 days) 2
   • For patients without urgent need for improvement: Either IVIG or corticosteroids 1
   • If using corticosteroids, options include:
     • Daily prednisolone (starting at 1-1.5 mg/kg/day)
     • Pulsed dexamethasone (40 mg daily for 4 days per month)
     • Pulsed IV methylprednisolone (1g daily for 3-5 days) 4

3. Response Assessment:

   • Evaluate clinical response after 2-4 weeks 2
   • If responding to treatment, continue with maintenance therapy 1
   • If no response or inadequate response, switch to alternative first-line therapy 2

Maintenance Therapy

• For patients responding to IVIG: Continue with regular IVIG infusions or transition to SCIG 3
• For patients responding to corticosteroids: Gradually taper to lowest effective dose 5
• Consider combination therapies for patients with partial response 1

Special Considerations

• Early treatment is crucial to prevent permanent nerve damage 3
• Corticosteroids may be more cost-effective but carry greater risk of long-term side effects 5
• Monthly high-dose oral dexamethasone may have fewer cosmetic side effects (less moon facies) and less sleeplessness compared to daily prednisolone 5
• Treatment should be initiated promptly after diagnosis to prevent permanent nerve damage 3

Monitoring

• Regular clinical assessment of muscle strength, sensory function, and disability 2
• Monitor for treatment-related adverse effects, particularly with corticosteroids 5
• Assess for remission and consider treatment tapering in stable patients 4