BACKGROUND: CIDP is a rare, difficult-to-diagnose, progressive disease caused by an abnormal autoimmune response to peripheral nerve myelin. Its predominant clinical manifestations are muscle weakness, sensory and other neurological impairments resulting in limited mobility, numbness, and impaired balance.
GOALS: To present an update of the literature on burden of disease, treatment options, and unmet needs in CIDP.
QUESTION: What is the disease burden of CIDP? What are the treatment options?
METHODS: Literature searches were conducted in PubMed, EMBASE, and the Cochrane Library to identify English-language articles reporting on the epidemiology, clinical and humanistic burdens, treatment patterns, and guidelines for CIDP, published between January 1, 2003 and September 27, 2018.
RESULTS: The searches identified 462 unique publications. Of these, 26 articles focusing on epidemiology (n=10), clinical and humanistic burden (n=7), and treatment patterns (n=9) of CIDP were included in data-extraction analysis. These studies reported prevalence as 1.61 to 12.00 per 100,000 people and incidence as 0.15 to 1.40 per 100,000 people, depending on the country and diagnostic criteria. Misdiagnosis was common (up to 47% of cases) because of the lack of standard diagnostic criteria and clinician’s knowledge and adherence to current guidelines. Quality of life was impaired due to mobility limitations, with the use of gait aids reported in 16%-27% of patients and wheelchair use in 10%-17% of patients. Intravenous immunoglobulin (IVIG) and corticosteroids are the most commonly used and recommended first-line treatments. Plasmapheresis is mostly recommended/prescribed as first-line therapy in severe cases. In real-world studies, more patients receiving IVIG responded to therapy than patients receiving corticosteroids (up to 80% vs up to 70%, respectively), and had fewer adverse events (4-16% vs 13-51%, respectively). Subcutaneous IG is approved as maintenance therapy only, is administered weekly over 1-2 consecutive days, and may involve several infusion sites per session.
CONCLUSIONS: CIDP is a rare disease, with relatively unknown pathogenesis, which, if left untreated, imposes a considerable burden on patients. CIDP diagnostic and treatment guidelines are underused during routine clinical care, resulting in misdiagnosis and undertreatment. There continues to be a need for increased awareness, timely and appropriate diagnosis, and treatment of CIDP. Baxalta US Inc. (a Takeda company) funded this study.
