目的:肥厚性硬脑膜炎(HP)是一种罕见的疾病,可引起局部或弥漫性炎性纤维化和硬脑膜增厚。抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)相关HP是HP的最常见形式。合并HP的AAV中耳炎(OMAAV)患者的ANCA阴性表型和疾病相关死亡率均较高。然而,很少有研究报道由于AAV/OMAAV引起的HP的影像学特征。因此,我们在本研究中调查了这个问题。
方法:这项回顾性研究包括2011年至2020年在我院诊断为HP的患者。年龄,性别,致病疾病,血清C反应蛋白(CRP)水平,和MRI数据是从医疗记录中收集的。我们根据病因疾病比较了MRI增强的位置。
结果:在18名HP患者中(平均年龄,64.1±2.6年;范围,33-77岁),10人(55.6%)为女性,12例(66.7%)被诊断为AAV/OMAAV,4人(22.2%)被诊断为特发性,2例(11.1%)被诊断为侵袭性曲霉菌乳突炎。11人(61.1%)有颅神经病变。面神经麻痹常见于AAV/OMAAV,外展神经麻痹常见于特发性HP。颅窝强化是HP患者最常见的表现,而内声道(IAC)增强仅见于AAV/OMAAV患者,而涉及海绵窦的HP仅见于特发性和乳突炎患者。
结论:涉及IAC的HP可能是诊断AAV/OMAAV的关键因素。
OBJECTIVE: Hypertrophic pachymeningitis (HP) is a rare disorder that causes localized or diffuse inflammatory fibrosis and thickening of the dura mater. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-related HP is the most frequent form of HP. Otitis media with AAV (OMAAV) patients with HP are associated with higher rates of both ANCA-negative phenotypes and disease-related mortality. However, few studies have reported the imaging characteristics of HP due to AAV/OMAAV. Therefore, we investigated this issue in the present study.
METHODS: This retrospective study included patients diagnosed with HP between 2011 and 2020 at our hospital. Age, sex, causative disease, serum C-reactive protein (CRP) level, and MRI data were collected from medical records. We compared the locations of MRI enhancement depending on the causative diseases.
RESULTS: Of the 18 included patients with HP (mean age, 64.1 ± 2.6 years; range, 33-77 years), 10 (55.6%) were female, 12 (66.7%) were diagnosed with AAV/OMAAV, four (22.2%) were diagnosed as idiopathic, two (11.1%) were diagnosed with invasive Aspergillus mastoiditis. Eleven (61.1%) had cranial neuropathies. Facial nerve paralysis was common in AAV/OMAAV, while abducent nerve paralysis was common in idiopathic HP. Cranial fossa enhancement was most common presentation in patients with HP, whereas inner acoustic canal (IAC) enhancement was seen only in patients with AAV/OMAAV, while HP involving the cavernous sinus was seen only in patients with idiopathic and mastoiditis.
CONCLUSIONS: HP involving the IAC may be a key factor in diagnosing AAV/OMAAV.