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Prevalence, Clinical Profiles, and Prognosis of CIDP in Japanese Nationwide Survey: Analyses of 1,257 Diagnosis-Confirmed Patients.
Aotsuka, Yuya; Misawa, Sonoko; Suichi, Tomoki; Shibuya, Kazumoto; Nakamura, Keigo; Kano, Hiroki; Otani, Ryo; Morooka, Marie; Ogushi, Moeko; Nagashima, Kengo; Sato, Yasunori; Kuriyama, Nagato; Kuwabara, Satoshi.
Afiliación
  • Aotsuka Y; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Misawa S; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Suichi T; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Shibuya K; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Nakamura K; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Kano H; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Otani R; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Morooka M; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Ogushi M; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Nagashima K; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Sato Y; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Kuriyama N; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
  • Kuwabara S; From the Department of Neurology (Y.A., S.M., T.S., K.S., K. Nakamura, H.K., R.O., M.M., M.O., S.K.), Graduate School of Medicine, Chiba University; School of Medicine (K. Nagashima, Y.S.), Keio University, Tokyo; and Shizuoka Graduate University of Public Health and Kyoto Prefectural University of
Neurology ; 102(6): e209130, 2024 Mar 26.
Article en En | MEDLINE | ID: mdl-38408295
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 1.51, 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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante Límite: Child / Female / Humans / Male / Middle aged País/Región como asunto: Asia Idioma: En Revista: Neurology Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante Límite: Child / Female / Humans / Male / Middle aged País/Región como asunto: Asia Idioma: En Revista: Neurology Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos