A nationwide survey of hypertrophic pachymeningitis in Japan

作者：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^*

来源：Journal of Neurology Neurosurgery and Psychiatry, 2014, 85(7): 732-739.

DOI：10.1136/jnnp-2013-306410

Objectives To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. %26lt;br%26gt;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. %26lt;br%26gt;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 %26apos;idiopathic%26apos; and 21 (13.2%) as %26apos;others%26apos;. 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. %26lt;br%26gt;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.

• 出版日期2014-7
• 单位河北医科大学