Fernandez M, Mordin M, Neighbors M, Tzivelekis S. A targeted literature review on the burden of Chronic Inflammatory Demyelinating Polyneuropathy. Poster presented at the 2019 American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) Conference; October 16, 2019. Austin, TX. [abstract] Muscle Nerve. 2019 Sep; p.112.


INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, progressive disease caused by an abnormal autoimmune response to peripheral nerve myelin. Its characteristic muscle weakness and sensory and other neurological impairments result in limited mobility, numbness, and impaired balance.

OBJECTIVE: To summarize the literature on burden and treatment options for CIDP.

METHODS: PubMed, EMBASE, and the Cochrane Library were searched for English-language articles reporting on epidemiology, clinical/humanistic burden, treatment patterns, and guidelines for CIDP, published January 1, 2003-September 27, 2018.

RESULTS: Of 462 unique publications identified, 26 focusing on epidemiology (n=10), clinical/humanistic burden (n=7), and treatment patterns (n=9) were analyzed. Prevalence is 1.61-12.00/100,000 people, and incidence is 0.15-1.40/100,000, depending on country and diagnostic criteria. Misdiagnosis is common (≤47% of cases) because of its similarities with other polyneuropathies. Quality of life is impaired due to mobility limitations, with use of gait aids reported in 16-27% of patients and wheelchair use in 10-17%. IVIg and corticosteroids are the most commonly recommended/prescribed first-line treatments, with plasma exchange mostly reserved for severe cases. In realworld studies, more patients receiving IVIg responded to therapy than patients receiving corticosteroids (≤80% versus ≤70%, respectively), and those receiving IVIg had fewer adverse events (4-16% versus 13-51%, respectively). Subcutaneous Ig, approved as maintenance therapy only, is administered weekly for 1-2 days, through several injection sites.

SUMMARY/CONCLUSION: CIDP is a rare disease, with relatively unknown pathogenesis, that can impose considerable burden on patients. Increased awareness, timely and correct diagnosis, and prompt treatment remain paramount.

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