From the Research
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an autoimmune neurological disorder that requires early treatment with first-line therapies such as intravenous immunoglobulin (IVIG), corticosteroids, or plasma exchange to prevent permanent nerve damage and improve outcomes. CIDP is characterized by progressive weakness and impaired sensory function in the arms and legs, occurring when the immune system mistakenly attacks the myelin sheath covering peripheral nerves, disrupting normal nerve signal transmission. Diagnosis typically involves nerve conduction studies, lumbar puncture showing elevated protein levels, and sometimes nerve biopsy.
First-line Treatment
- IVIG at 2g/kg divided over 2-5 days, with maintenance doses of 1g/kg every 3-4 weeks
- Plasma exchange (removing antibodies from blood)
- Corticosteroids like prednisone at 60-100mg daily with gradual taper, as suggested by 1
Second-line Treatment
- Azathioprine (2-3mg/kg/day)
- Mycophenolate mofetil (1-2g/day)
- Rituximab (375mg/m² weekly for 4 weeks), as mentioned in 2 Physical therapy is crucial to maintain strength and function. CIDP is chronic but treatable, with about 70-80% of patients responding to immunomodulatory therapy, as shown in 3. Unlike Guillain-Barré syndrome which is acute and monophasic, CIDP progresses over at least 8 weeks and can follow a relapsing-remitting or steadily progressive course.
Key Considerations
- Early treatment significantly improves outcomes and prevents permanent nerve damage
- The choice of treatment should be individualized based on costs, availability, and potential adverse effects, as discussed in 4
- Maintenance therapy is often necessary to prevent relapse, with options including IVIG, corticosteroids, or immunosuppressants, as mentioned in 3 and 5