MPO-ANCA相关性肥厚性硬脑膜炎合并肉芽肿性血管炎临床分析

Clinical analysis of myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic cranial pachymeningitis with granulomatosis with polyangiitis

目的探讨髓过氧化物酶抗中性粒细胞胞质抗体(MPO-ANCA)相关性肥厚性硬脑膜炎(HCP)合并肉芽肿性血管炎(GPA)的临床特点、治疗及预后。方法回顾性分析2016-06—2020-06郑州大学第一附属医院神经内科住院治疗的MPO-ANCA相关性HCP合并GPA患者的临床资料。结果5例患者均为亚急性或慢性起病,病程1个月~3 a,首发症状主要为头痛5例,脑神经受损2例。首次入院时所有患者核周型-抗中性粒细胞胞浆抗体(p-ANCA)阳性,MPO-ANCA浓度及非特异性炎症指标升高,胞质型抗中性粒细胞胞质抗体(c-ANCA)和蛋白酶3抗中性粒细胞胞质抗体(PR3-ANCA)正常,2例尿常规隐血阳性,24 h尿蛋白总量升高,颅脑MRI增强扫描均可见不同部位的硬脑膜强化及鼻窦炎,3例伴乳突炎。5例患者糖皮质激素治疗均有效,4例病情反复,3例联用免疫抑制剂治疗后症状缓解。结论MPO-ANCA相关性HCP合并GPA病例多伴鼻窦炎、乳突炎、尿隐血、尿蛋白等,应早期给予糖皮质激素联合免疫抑制剂治疗,避免疾病向全身型发展。

Objective To investigate clinical characteristics,treatment and prognosis of myeloperoxidase antineutrophil cytoplasmic antibody(MPO-ANCA)associated hypertrophic cranial pachymeningitis(HCP)with granulomatosis with polyangiitis(GPA).Methods The clinical data from June 2016 to June 2020 of the inpatients of MPO-ANCA associated HCP with GPA in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.Results The onset of MPO-ANCA associated HCP with GPA was subacute or chronic with the course of a month to 3 years.The main clinical symptoms were headache(5/5)and injury of cranial nerves(2/5).At the time of the first admission,all patients were positive for nucleocyocytic plasma antibodies(p-ANCA),showing increased levels of MPO-ANCA and inflammatory markers,while cytoplasmic antineutrophil antibodies(c-ANCA)and protease 3 antineutrophil cytoplasmic antibodies(PR3-ANCA)were negative,and there were 2 cases of positive for haematuria on urinalysis and total 24h urine protein increasing.Different parts of dura,obviously enhanced,sinusitis(5/5),mastoiditis(3/5)were demonstrated in magnetic resonance imaging(MRI).Glucocorticoid was effective for 5 patients.But 3 patients relapsed and symptoms were alleviated after treatment with immunosuppressive agents.Conclusion MPO-ANCA associated HCP with GPA are often accompanied by sinusitis,mastitis,haematuria,or urine protein.Glucocorticoid and immunosuppressive agents should be used during the early stages for avoiding disease developing a generalized disease state.