Contemporary challenges in the diagnosis and management of chronic inflammatory demyelinating polyneuropathy

Introduction: Despite extensive research, multiple inter-related diagnostic and management challenges remain for chronic inflammatory demyelinating polyneuropathy (CIDP). Areas Covered: A literature review was performed on diagnosis and treatment in CIDP. The clinical features and disease course were evaluated. Investigative techniques, including electrophysiology, cerebrospinal fluid examination, neuropathology, imaging, and neuroimmunology, were considered in relation to technical aspects, sensitivity, specificity, availability, and cost. Available evidenced-based treatments and those with possible efficacy despite lack of evidence, were considered, as well as current methods for evaluation of treatment effects. Expert Opinion: CIDP remains a clinical diagnosis, supported first and foremost by electrophysiology. Other investigative techniques have limited impact. Most patients with CIDP respond to available first-line treatments and immunosuppression may be efficacious in those who do not. Consideration of the natural history and of the high reported remission rate, of under-recognized associated disabling features, of treatment administration modalities and assessment methods, require enhanced attention.