A nationwide survey of hypertrophic pachymeningitis in Japan

Tomomi Yonekawa, Hiroyuki Murai, Satoshi Utsuki, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Mari Yoshida, Susumu Kusunoki, Kiyomi Sakata, Kiyotaka Fujii, Jun-Ichi Kira

Research output: Contribution to journalArticle

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Abstract

Objectives: To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. Methods: The study began with a preliminary survey to determine the approximate number of HP patients diagnosed from 1 January 2005 to 31 December 2009, and was followed by a questionnaire survey for clinical and laboratory findings. HP was defined as a condition with thickening of the cranial or spinal dura mater with inflammation, evidenced by MRI or histology. Results: Crude HP prevalence was 0.949/100 000 population. The mean age at onset was 58.3±15.8 years. Among 159 cases for whom detailed data were collated, antineutrophil cytoplasmic antibody (ANCA)-related HP was found in 54 cases (34.0%) and IgG4/multifocal fibrosclerosis (MFS)-related HP in 14 cases (8.8%). Seventy cases (44.0%) were classified as 'idiopathic' and 21 (13.2%) as 'others'. ANCA-related HP cases showed a female preponderance, a higher age of onset, and higher frequencies of otological symptoms and elevated systemic inflammatory biomarkers, but lower frequencies of diplopia compared with idiopathic HP. IgG4/MFS-related HP cases showed a marked male predominance; all had cranial HP while none had isolated spinal HP or decreased sensation. Conclusions: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

Original languageEnglish
Pages (from-to)732-739
Number of pages8
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume85
Issue number7
DOIs
Publication statusPublished - Jan 1 2014

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Meningitis
Japan
Antineutrophil Cytoplasmic Antibodies
Immunoglobulin G
Surveys and Questionnaires
Age of Onset
Dura Mater
Diplopia
Histology
Biomarkers

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

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A nationwide survey of hypertrophic pachymeningitis in Japan. / Yonekawa, Tomomi; Murai, Hiroyuki; Utsuki, Satoshi; Matsushita, Takuya; Masaki, Katsuhisa; Isobe, Noriko; Yamasaki, Ryo; Yoshida, Mari; Kusunoki, Susumu; Sakata, Kiyomi; Fujii, Kiyotaka; Kira, Jun-Ichi.

In: Journal of Neurology, Neurosurgery and Psychiatry, Vol. 85, No. 7, 01.01.2014, p. 732-739.

Research output: Contribution to journalArticle

Yonekawa, Tomomi ; Murai, Hiroyuki ; Utsuki, Satoshi ; Matsushita, Takuya ; Masaki, Katsuhisa ; Isobe, Noriko ; Yamasaki, Ryo ; Yoshida, Mari ; Kusunoki, Susumu ; Sakata, Kiyomi ; Fujii, Kiyotaka ; Kira, Jun-Ichi. / A nationwide survey of hypertrophic pachymeningitis in Japan. In: Journal of Neurology, Neurosurgery and Psychiatry. 2014 ; Vol. 85, No. 7. pp. 732-739.
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AU - Murai, Hiroyuki

AU - Utsuki, Satoshi

AU - Matsushita, Takuya

AU - Masaki, Katsuhisa

AU - Isobe, Noriko

AU - Yamasaki, Ryo

AU - Yoshida, Mari

AU - Kusunoki, Susumu

AU - Sakata, Kiyomi

AU - Fujii, Kiyotaka

AU - Kira, Jun-Ichi

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N2 - Objectives: To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. Methods: The study began with a preliminary survey to determine the approximate number of HP patients diagnosed from 1 January 2005 to 31 December 2009, and was followed by a questionnaire survey for clinical and laboratory findings. HP was defined as a condition with thickening of the cranial or spinal dura mater with inflammation, evidenced by MRI or histology. Results: Crude HP prevalence was 0.949/100 000 population. The mean age at onset was 58.3±15.8 years. Among 159 cases for whom detailed data were collated, antineutrophil cytoplasmic antibody (ANCA)-related HP was found in 54 cases (34.0%) and IgG4/multifocal fibrosclerosis (MFS)-related HP in 14 cases (8.8%). Seventy cases (44.0%) were classified as 'idiopathic' and 21 (13.2%) as 'others'. ANCA-related HP cases showed a female preponderance, a higher age of onset, and higher frequencies of otological symptoms and elevated systemic inflammatory biomarkers, but lower frequencies of diplopia compared with idiopathic HP. IgG4/MFS-related HP cases showed a marked male predominance; all had cranial HP while none had isolated spinal HP or decreased sensation. Conclusions: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

AB - Objectives: To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. Methods: The study began with a preliminary survey to determine the approximate number of HP patients diagnosed from 1 January 2005 to 31 December 2009, and was followed by a questionnaire survey for clinical and laboratory findings. HP was defined as a condition with thickening of the cranial or spinal dura mater with inflammation, evidenced by MRI or histology. Results: Crude HP prevalence was 0.949/100 000 population. The mean age at onset was 58.3±15.8 years. Among 159 cases for whom detailed data were collated, antineutrophil cytoplasmic antibody (ANCA)-related HP was found in 54 cases (34.0%) and IgG4/multifocal fibrosclerosis (MFS)-related HP in 14 cases (8.8%). Seventy cases (44.0%) were classified as 'idiopathic' and 21 (13.2%) as 'others'. ANCA-related HP cases showed a female preponderance, a higher age of onset, and higher frequencies of otological symptoms and elevated systemic inflammatory biomarkers, but lower frequencies of diplopia compared with idiopathic HP. IgG4/MFS-related HP cases showed a marked male predominance; all had cranial HP while none had isolated spinal HP or decreased sensation. Conclusions: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

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