Chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disorder of the peripheral nervous system characterized by progressive or relapsing weakness and impaired sensory function in the legs and arms. The disease results from damage to the myelin sheath — the protective fatty covering that insulates peripheral nerves — caused by an abnormal autoimmune response. CIDP is sometimes considered the chronic counterpart of Guillain-Barré syndrome (acute inflammatory demyelinating polyneuropathy). Symptoms typically develop over at least eight weeks and include symmetric proximal and distal muscle weakness, numbness, tingling, and pain in the extremities, reduced or absent deep tendon reflexes, fatigue, and difficulty walking. Some patients experience a relapsing-remitting course, while others have a steadily progressive decline. CIDP primarily affects the peripheral nervous system, including motor and sensory nerves throughout the body. Electrodiagnostic studies (nerve conduction studies and electromyography) typically reveal evidence of demyelination, such as prolonged distal latencies, reduced conduction velocities, conduction block, and temporal dispersion. Cerebrospinal fluid analysis often shows elevated protein levels without a significant increase in white blood cells (albuminocytologic dissociation). Nerve biopsy, though not always required for diagnosis, may demonstrate segmental demyelination and inflammatory infiltrates. Several effective treatments are available for CIDP. First-line therapies include intravenous immunoglobulin (IVIg), subcutaneous immunoglobulin, and corticosteroids (such as oral prednisone or pulsed intravenous methylprednisolone). Plasma exchange (plasmapheresis) is also an established treatment option. For patients who do not respond adequately to these therapies, immunosuppressive agents such as azathioprine, mycophenolate mofetil, or rituximab may be considered. Early diagnosis and treatment are important, as prolonged demyelination can lead to secondary axonal damage and irreversible disability. With appropriate treatment, many patients experience significant improvement, though long-term maintenance therapy is often required.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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