What is the recommended treatment for a patient with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

For newly diagnosed CIDP, initiate intravenous immunoglobulin (IVIG) 2g/kg divided over 2-5 days as first-line therapy, as it achieves approximately 80% initial response rates with favorable side effects compared to other options. 1

Initial Treatment Selection Based on Disease Severity

For severe or rapidly progressive CIDP:

• Administer pulse methylprednisolone 1g IV daily for 3-5 days PLUS IVIG 2g/kg over 5 days (0.4 g/kg/day), particularly when patients present with acute inflammatory demyelinating polyneuropathy-type features. 1
• Hospital admission is warranted for severe symptoms with functional impairment. 1

For mild to moderate disease:

• IVIG 2g/kg over 2-5 days remains the preferred initial therapy due to rapid onset and tolerability. 1
• Alternatively, oral prednisone 1 mg/kg daily with gradual taper over 4-6 weeks can be used, particularly in young, otherwise healthy patients. 1, 2

For elderly patients or those with comorbidities (diabetes, obesity, hypertension):

• Plasma exchange may be preferred as first-line therapy over corticosteroids to avoid steroid-related complications. 2
• However, plasma exchange requires specialized centers, vascular access, and has transient effects limiting its use for long-term maintenance. 2

Critical Timing Considerations

• Do not delay treatment beyond 2 weeks, as this correlates with severe neurological deficit and poor outcomes. 1
• Initiate treatment as early as possible—shorter disease duration before treatment correlates with better response to therapy. 3
• Approximately 40% of patients do not improve in the first 4 weeks but may still benefit from continued therapy, so do not assume treatment failure prematurely. 1

Maintenance and Long-Term Management

After initial response to IVIG or plasma exchange:

• Transition to oral prednisone for long-term maintenance, as IVIG is prohibitively expensive for sustained use and plasma exchange is impractical for chronic administration. 2
• Prednisone dosing should be tapered very gradually—rapid tapering increases relapse risk. 3
• A period of stability is needed before attempting to taper corticosteroids; most patients achieve maximal improvement after 4 weeks of high-dose therapy. 3

For treatment-related fluctuations (TRFs):

• Approximately 6-10% of patients experience disease progression within 2 months following initial treatment-induced improvement. 1
• Repeat the full course of IVIG or plasma exchange, as TRFs indicate the treatment effect has worn off while inflammation persists. 1

Second-Line Therapy for Refractory Cases

When first-line therapies fail or patients cannot tolerate them:

• Rituximab should be considered in consultation for patients with limited or no improvement after first-line therapy. 1
• Ciclosporin 3 mg/kg/day (plasma trough 100-150 ng/ml) is effective for intractable CIDP requiring repeated IVIG, with potential for 2-year remission maintenance. 4
• Other immunosuppressants include azathioprine, cyclophosphamide, or mycophenolate mofetil for maintenance therapy or when symptoms persist after 4 weeks. 1, 2

Neuropathic Pain Management

First-line pain medications:

• Pregabalin 300-600 mg/day is supported by high-quality evidence for neuropathic pain in CIDP. 5
• Gabapentin 900-3600 mg/day is effective, though not FDA-approved specifically for CIDP pain. 5
• Duloxetine 60-120 mg/day is FDA-approved for neuropathic pain with proven efficacy. 5

Second-line pain options:

• Tricyclic antidepressants (amitriptyline 25-75 mg/day) are effective but have dose-limiting anticholinergic effects, especially in patients ≥65 years—start at 10 mg/day in older patients and titrate slowly. 5
• Tramadol 200-400 mg/day can be considered but carries addiction risk; avoid long-term opioids due to dependence, hypogonadism, and abuse potential. 5

Pain medication titration:

• Start gabapentinoids and SNRIs at low doses with gradual titration to minimize sedation, dizziness, and peripheral edema. 5

Diagnostic Confirmation Requirements Before Treatment

• Nerve conduction studies demonstrating demyelinating features are necessary for diagnosis. 1
• Cerebrospinal fluid analysis showing cytoalbuminologic dissociation (elevated protein with normal cell count) supports the diagnosis. 1
• Exclude CNS involvement through neuroimaging when focal signs, gait disturbance, or increased reflexes are present. 1

Common Pitfalls to Avoid

• Do not perform plasmapheresis immediately after IVIG administration, as it removes the therapeutic immunoglobulin. 1
• Do not taper corticosteroids too rapidly—this significantly increases relapse risk, with 8 of 9 patients in one study developing 26 relapses when tapering was attempted. 3
• Do not assume poor prognosis based solely on CSF protein levels or cranial nerve involvement—these do not correlate with treatment response. 3
• Recognize that global distribution of weakness, muscle atrophy, and positive Babinski sign predict poor prognosis and may require more aggressive therapy. 3

Comparative Efficacy of Corticosteroid Regimens

• Daily oral prednisolone, pulsed oral dexamethasone, and pulsed intravenous methylprednisolone show equivalent efficacy (60% response rate) and safety profiles with no significant differences between regimens. 6
• Among responders, 61% achieve remission with 55% probability of 5-year remission, regardless of corticosteroid regimen used. 6
• Adverse events leading to treatment change occur in only 8% of patients, with serious adverse events being rare. 6