From the Research
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare autoimmune disorder that affects the peripheral nerves, causing progressive weakness and impaired sensory function in the arms and legs, and is characterized by immune-mediated damage to the myelin sheath of peripheral nerves, disrupting nerve signal transmission, as described in the study by 1.
Definition and Pathophysiology
CIDP is an immune-mediated neuropathy that responds to various immunosuppressive treatments, with the pathophysiology involving inflammatory demyelination that manifests as slowed conduction velocities, temporal dispersion, and conduction block on nerve conduction studies, as noted in the study by 1.
Clinical Features
The commonest form of CIDP causes more or less symmetrical progressive or relapsing weakness affecting proximal and distal muscles, with large-fiber abnormalities (weakness and ataxia) predominating, whereas small-fiber abnormalities (autonomic and pain) are less common, as described in the study by 1.
Diagnosis
The diagnosis of CIDP should be based on clinical features, electrophysiological studies, and nerve biopsy, with other neuropathies resembling CIDP, and it is essential to identify these to ensure correct treatment, as noted in the study by 1.
Treatment
The treatment of CIDP typically begins with corticosteroids like prednisone, intravenous immunoglobulin (IVIG), or plasma exchange, with IVIG often preferred as first-line therapy due to its favorable side effect profile, as described in the study by 2.
- The use of corticosteroids, such as prednisone, has been shown to be effective in the treatment of CIDP, with a study by 2 demonstrating that corticosteroids are beneficial, but long-term use causes serious side effects.
- IVIG is often used as first-line therapy, with maintenance doses of 1g/kg every 3-4 weeks, adjusted based on response, as noted in the study by 1.
- Plasma exchange is also used as a treatment option, typically 5 exchanges over 2 weeks, as described in the study by 3.
Long-term Management
For long-term management, immunosuppressants like azathioprine, mycophenolate mofetil, or rituximab may be added as steroid-sparing agents, as noted in the study by 4.
Monitoring and Follow-up
Patients should be monitored regularly with neurological examinations, electrophysiological studies, and disability assessments to evaluate treatment response, as described in the study by 1.