Prevalence, Clinical Profiles, and Prognosis of CIDP in Japanese Nationwide Survey
Analyses of 1,257 Diagnosis-Confirmed Patients
Abstract
Background and Objectives
To investigate the current epidemiology, clinical profile, and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) using a nationwide survey in Japan.
Methods
We conducted a nationwide survey using an epidemiologic method established in 2021. Questionnaire sheets were sent to the hospital departments of neurology and pediatric neurology throughout Japan. A primary questionnaire was used to determine the number of patients and their prevalence, and a second questionnaire was used to collect detailed clinical information.
Results
The primary survey showed that the estimated number of patients with CIDP was 4,180, with a prevalence of 3.3 per 100,000 persons. In the secondary survey, detailed clinical data were available for 1,257 patients. The male-to-female ratio was 1.5:1, and the median age at onset was 52 years. Typical CIDP was the most frequent subtype (52%), followed by distal (17%) and multifocal/focal CIDP (17%). Initial treatments included immunoglobulin therapy (72%), corticosteroids (15%), and others (13%). Among patients with CIDP, 78% had a progressive/relapsing course, 14% did not respond to first-line treatments, and 18% could not walk independently at the last visit. Among the subtypes, typical CIDP had the most severe disability before treatment (44% of patients could not walk independently). However, they showed a more favorable response to treatment than those with distal or multifocal CIDP. In the subgroup analyses, logistic regression analyses showed that younger age at onset, no muscle atrophy, and abnormal median-normal sural sensory nerve responses were associated with a higher probability of independent walking.
Discussion
Our study represents the largest cohort study on CIDP to demonstrate the current epidemiologic and clinical status of CIDP in Japan. Clinical subtypes seem to be associated with different treatment responses and outcomes; therefore, an appropriate treatment strategy according to the pathophysiology of each subtype is required to improve the prognosis of CIDP.
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Supplementary Material
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© 2024 American Academy of Neurology.
Publication History
Received: August 7, 2023
Accepted: November 20, 2023
Published online: February 26, 2024
Published in print: March 26, 2024
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Study Funding
This work was partly supported by the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunologic Diseases) from the Ministry of Health, Labour, and Welfare of Japan (20FC1030).
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- Diagnostic testing and treatment in Japanese patients with a diagnosis code for chronic inflammatory demyelinating polyradiculoneuropathy: A claims database analysis, Clinical and Experimental Neuroimmunology, (2024).https://doi.org/10.1111/cen3.12813
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- Early differential diagnosis between acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy in children: Clinical factors and routine biomarkers, European Journal of Paediatric Neurology, 53, (25-32), (2024).https://doi.org/10.1016/j.ejpn.2024.09.005
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