Navigating Chronic Inflammatory Demyelinating Polyneuropathy: Managing a Rare Autoimmune Disorder

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder marked by chronic inflammation and progressive damage to the myelin sheath of peripheral nerves, causing muscle weakness, sensory loss, and diminished reflexes. It presents in various forms, including typical CIDP, distal acquired demyelinating symmetric neuropathy (DADS), and multifocal variants. Diagnosis involves clinical assessments, electrodiagnostic studies, and cerebrospinal fluid analysis, with imaging or nerve biopsy reserved for complex cases. Treatment focuses on immunomodulatory therapies like intravenous immunoglobulin (IVIg), corticosteroids, or plasmapheresis to manage symptoms and limit nerve damage.

CIDP affects 0.67 to 10.3 per 100,000 people, more often men, typically with onset between ages 40 and 60. While treatments are effective, challenges remain with adherence, relapses, and variable patient responses. Research is advancing with emerging biologics such as neonatal Fc receptor (FcRn) inhibitors and complement inhibitors. Prognosis varies, with some patients achieving remission, though ongoing management is crucial to prevent relapses and disability. Continued research aims to enhance diagnostics and develop therapies tailored to individual immune responses.

Reference: Gonzalez Caldito N, Habib AA. Diagnosis of chronic inflammatory demyelinating polyneuropathy. Practical Neurology (US). 2024;23(3):19-25.