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

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

First-line treatment for CIDP consists of three equally effective options: corticosteroids, intravenous immunoglobulin (IVIG), or plasma exchange, with the choice depending on disease severity, patient comorbidities, and treatment accessibility. 1, 2

First-Line Treatment Options

Corticosteroids

• Pulse methylprednisolone 1g IV daily for 3-5 days is recommended for severe or progressing symptoms, followed by a taper over at least 4-6 weeks 3, 1
• Oral corticosteroids alone or with immunosuppressive therapy achieve 60-75% response rates in peripheral neuropathies 3
• Corticosteroids are particularly useful when cost or venous access is a limiting factor 2, 4

Intravenous Immunoglobulin (IVIG)

• Standard dosing is 2g/kg administered over 5 days (0.4 g/kg/day) 3, 1
• IVIG is well-established as first-line therapy and at least two-thirds of CIDP patients require ongoing infusions for several years 2, 5
• For severe cases with acute inflammatory demyelinating polyneuropathy-type presentation, consider combining pulse corticosteroids PLUS IVIG 3, 1

Subcutaneous Immunoglobulin (SCIg)

• SCIg has been recently approved and provides an alternative to IVIG with improved patient independence and tolerability 2, 4, 6
• Long-term SCIg treatment (up to 7 years) maintains stable or improved strength and motor function in pre-selected CIDP patients 5
• SCIg is particularly valuable for patients requiring chronic maintenance therapy who prefer home-based treatment 5, 6

Plasma Exchange

• Plasma exchange is an established first-line option, particularly effective in severe cases 3, 2, 4
• Important caveat: Plasmapheresis immediately after IVIG will remove immunoglobulin, so timing must be carefully considered 3

Treatment Algorithm for Severity

Mild to Moderate Disease (Grade 1-2)

• Initiate oral corticosteroids (prednisone 1 mg/kg daily) with gradual taper over 4-6 weeks 3
• Alternative: IVIG 2g/kg over 5 days if corticosteroid contraindications exist 1, 2

Severe or Rapidly Progressive Disease (Grade 3-4)

• Pulse methylprednisolone 1g IV daily for 3-5 days PLUS IVIG 2g/kg over 5 days 3, 1
• Consider plasma exchange if no improvement or symptoms worsen after 3 days 3
• Hospital admission is warranted for severe symptoms with functional impairment 3

Second-Line and Refractory Treatment

Immunosuppressive Agents

• Rituximab should be considered in consultation for patients with limited or no improvement after first-line therapy 3, 1
• Other immunosuppressants include methotrexate, azathioprine, or mycophenolate mofetil for maintenance therapy or when symptoms do not resolve after 4 weeks 3, 2
• These agents are widely used in clinical practice despite limited randomized trial evidence 7

Treatment-Related Fluctuations

• Approximately 6-10% of patients experience treatment-related fluctuations (TRFs), defined as disease progression within 2 months following initial treatment-induced improvement 3
• Repeat the full course of IVIG or plasma exchange for TRFs, as this indicates the treatment effect has worn off while inflammation persists 3

Acute-Onset CIDP

• In ~5% of patients, three or more TRFs and/or clinical deterioration ≥8 weeks after onset suggests acute-onset CIDP rather than Guillain-Barré syndrome 3
• These patients require long-term maintenance immunotherapy rather than single-course treatment 3, 1

Diagnostic Confirmation Requirements

Before initiating treatment, confirm:

• Progressive bilateral weakness with areflexia developing over more than 2 months (key distinguishing feature from acute GBS) 1, 6
• Cerebrospinal fluid showing cytoalbuminologic dissociation (elevated protein with normal cell count) 1
• Nerve conduction studies demonstrating demyelinating features 3, 1
• Exclusion of CNS involvement through neuroimaging when focal signs, gait disturbance, or increased reflexes are present 3

Special Considerations

IgM-Associated Neuropathy

• 50% of patients with IgM MGUS and peripheral neuropathy have anti-myelin-associated glycoprotein (MAG) antibodies 1
• Rituximab is effective in a subset of these patients 1, 2

Nodal/Paranodal Antibody-Associated CIDP

• Neuropathies with antibodies to contactin-1, contactin-associated protein 1, or neurofascin 155 present with unique features 7, 6
• These variants are often refractory to IVIG therapy and may require alternative immunosuppressive approaches 6

Monitoring and Maintenance

• Most patients (67%) maintain stable nerve conduction parameters over 7 years with appropriate treatment 3
• Long-term maintenance therapy is typically required, as discontinuation often leads to relapse 2, 5
• Regular assessment using Medical Research Council sum-score and Overall Neuropathy Limitation Scale tracks treatment response 5

Common Pitfalls to Avoid

• Do not delay treatment beyond 2 weeks, as this is associated with severe neurological deficit and poor outcomes 3
• Do not perform plasmapheresis immediately after IVIG administration, as it removes the therapeutic immunoglobulin 3
• Do not assume treatment failure if progression continues in the first 4 weeks—40% of patients do not improve initially but may still benefit from therapy 3
• Do not discontinue maintenance therapy prematurely, as most patients require years of treatment to prevent relapse 2, 5