江汉大学学报（自然科学版）

目的探讨MPO-ANCA阳性相关性肥厚性硬脑膜炎的临床特征及治疗方案。方法报道1例MPO-ANCA阳性的肥厚性硬脑膜炎伴肺动脉血栓的患者,并结合文献进行分析。结果患者就诊时表现为视力下降,伴有头痛,入院后查血D-二聚体7.09 mg/L FEU(<0.5),红细胞沉降率33 mm/h(<15),抗MPO抗体137.3 CU(<20.0),抗蛋白酶3抗体22.7 CU(<20.0),肺纵膈三维CT平扫+增强示双侧肺动脉广泛血栓形成,肺动脉CTA示肺动脉多发栓塞,颅脑磁共振示双侧额颞部脑膜增厚强化,海绵窦、眶尖、咽旁间隙(右侧为著)及右侧岩上/下窦条片状强化影,所及双侧视神经局部显示较纤细(右侧为著),颅底脑膜增厚局部稍明显/疑稍显厚,诊断为MPO-ANCA阳性的肥厚性硬脑膜炎,经免疫、抗凝治疗后症状好转。结论 MPO-ANCA阳性相关性肥厚性硬脑膜炎临床表现具有异质性,大多数患者出现慢性头痛,伴或不伴颅脑神经麻痹、小脑共济失调、癫痫发作、脊髓病、视力障碍等神经学表现。在临床中,发现头痛、多颅神经受累,并且伴鼻窦炎、中耳炎患者,需考虑MPO-ANCA阳性的肥厚性硬脑膜炎的可能。

Objective To investigate the clinical characteristics and treatment of MPOANCA positive associated hypertrophic cranial pachymeningitis. Methods A case of MPOANCA positive hypertrophic cranial pachymeningitis with extensive pulmonary arterial thrombosis was reported and analyzed with the literature. Results The patient presented with impaired vision and headache. After admission,blood D-dimer 7. 09 mg/L FEU( < 0. 5),ESR 33 mm/h( < 15),anti-MPO antibody 137. 3 CU( < 20. 0),anti-protease 3 antibody 22. 7 CU( < 20. 0). The three-dimensional CT scan of the mediastinum plus enhancement shows extensive thrombosis of both pulmonary arteries. Pulmonary artery CTA showed multiple pulmonary embolisms. Cerebral MRI showed in bilateral frontal-temporal dura thickening reinforcement,cavernous sinus,orbit,pharynx side clearance(right),and on the right side of the rock/article sinus flake reinforced under the shadow,and double side nerve local display was fine(right),the dura thickening of the skull base slightly obvious local/doubt slightly thick,diagnosis of MPO ANCA positive hypertrophic pachymeningitis,symptoms improved after immunization, anticoagulant therapy. Conclusion The clinical manifestations of MPO-ANCA positive hypertrophic cranial pachymeningitis are heterogeneous. Most patients have chronic headaches,with or without craniocerebral nerve palsy, cerebellar ataxia, epileptic seizure, myelopathy, visual impairment, and other neurological manifestations. In clinical practice,the possibility of MPO-ANCA positive hypertrophic cranial pachymeningitis should be considered in patients with headaches,multiple cranial nerve involvement,and sinusitis and otitis media.