Abstract
Chronic inflammatory demyelination polyradiculoneuropathy (CIDP) is a rare autoimmune neuropathy generated by cellular and humoral immune responses. Its course can be chronic, progressive, monophasic, or relapsing-remitting. Misdiagnosis and inappropriate therapy are common in CIDP. Given the scarcity of integrative information, we aimed to briefly summarize the epidemiology, pathophysiology, clinical phenotypes, diagnostic tools, and diagnostic criteria and provide a systematic diagnostic approach. We reviewed articles on Medline (PubMed) from 2018 to 2023, using Google Scholar to summarize the topics. The results are presented as a narrative review, in accordance with recommendations of the Scale for the Assessment of Narrative Review Articles (SANRA) guidelines. The included evidence showed that CIDP is a challenging neuropathy to diagnose and treat. Pathologic factors initiating typical CIDP and atypical CIDP are still clearly unknown. CIDP is diagnosed using the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria, which combine clinical features with electrophysiological evidence of demyelination. However, some patients need to fulfill the requirements. Another challenge is monitoring the disease progression and recognizing patients who do not respond to evidence-based first-line therapy to individualize their treatment. Based on the evidence, we conclude that 2021 EFNS/PNS guidelines allow for a more accurate diagnosis and treatment of CIDP and its variants. New diagnostic tools and molecular approaches are helpful in the diagnosis process but cannot replace clinical and electrodiagnostic criteria.