■肥厚性硬脑膜炎(HP)是一种病因多样的疾病,包括自身免疫的,与抗中性粒细胞胞浆抗体或免疫球蛋白G4相关。
■一名65岁女性,有系统性动脉高血压病史,表现出强烈的进行性头痛。通过核磁共振(NMR)研究观察到HP和半球血管源性水肿。在头痛前的六个月里,她患有进行性听力损失,这归因于年龄。硬脑膜活检显示坏死性血管炎伴周围炎性浸润,由上皮样细胞和多核巨细胞组成,和丰富的嗜酸性粒细胞。最终诊断为HP伴嗜酸性肉芽肿伴多血管炎(EGPA)。
■患者有嗜酸性肉芽肿伴多血管炎(EGPA)组织学,ANCA阴性血清学和HP。这个案例很重要,因为它表明EGPA似乎有一系列临床疾病,包括血清学阴性的HP,和双侧感音神经性听力损失。
■我们面临着广泛的EGPA,打破只有系统性参与的范式。
结论:肥厚性硬脑膜炎(HP)有多种病因;如果系统检查对诊断没有帮助,脑膜活检是必要的.这是HP的首例病例报告,与嗜酸性肉芽肿性多血管炎(EGPA)相关,和ANCA阴性血清学。EGPA可能是一系列主要的全身性疾病,但有些病例可能有组织学证据,没有系统背景或血清学阳性。
UNASSIGNED: Hypertrophic pachymeningitis (HP) is a disease with diverse aetiologies, including the autoimmune one, either associated with antineutrophil cytoplasmic antibodies or immunoglobulin G4.
UNASSIGNED: A 65-year-old woman with a history of systemic arterial hypertension, presented with intense progressive headaches. HP and hemispheric vasogenic oedema were observed by nuclear magnetic resonance (NMR) study. During the six months before the headache, she had developed progressive hearing loss which she attributed to age. A biopsy of dura mater showed necrotising vasculitis with peripheral inflammatory infiltrate, made up of accumulations of epithelioid cells and multinucleated giant cells, and abundant eosinophils. A final diagnosis of HP with eosinophilic granulomatosis with polyangiitis (EGPA) was made.
UNASSIGNED: The patient had eosinophilic granulomatosis with polyangiitis (EGPA) histology, ANCA-negative serology and HP. This case is important because it shows that EGPA seems to have a spectrum of clinical diseases, including HP with negative serology, and bilateral sensorineural hearing loss.
UNASSIGNED: We are facing a wide spectrum of EGPA, breaking the paradigm of only systemic involvement.
CONCLUSIONS: Hypertrophic pachymeningitis (HP) has several aetiologies; if the systemic investigation is not contributory to a diagnosis, a meningeal biopsy is necessary.This is the first case report of HP, associated with eosinophilic granulomatosis with polyangiitis (EGPA), and ANCA-negative serology.EGPA is probably a spectrum of diseases with predominant systemic involvement, but there may be cases where there is histological evidence, without the systemic context or positive serology.