髓过氧化物酶抗中性粒细胞胞质抗体阳性的肥厚性硬脑膜炎临床分析

目的 探讨髓过氧化物酶抗中性粒细胞胞质抗体阳性(MPO-ANCA)的肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床特点及诊治方法.方法 回顾性分析2018—2020年河南省人民医院收治的7例MPO-ANCA阳性的HCP笔者的临床资料.结果 7例患者中男6例,女1...

Clinical analysis of MPO-ANCA associated hypertrophic pachymeningitis

Objective To explore the clinical features,diagnosis and treatment methods of MPO-ANCA associated with hypertrophic pachymeningitis.Methods The clinical data of 7 MPO-ANCA patients were retrospectively analyzed who were admitted to Henan Provincial People’s Hospital from 2018 to 2020.Results All these 7 patients,6 males and 1 female with an average age of onset of 63 years old,had headache onset.Five patients demonstrated cranial nerve involvement,2 patients demonstrated cranial otitis media,and 2 patients demonstrated cranial mastoiditis.None of them had lung or kidney damage.At the preliminary visit,C-reactive protein increased,erythrocyte sedimentation rate increased,MPO-ANCA was positive,PR3-ANCA was negative,and all patients had CSF protein increased.All patients have increasing thickness and strength of the dura mater.Glucocorticoid treatment is considered to ameliorate the symptoms.Three patients were initially treated with hormones alone,and the disease relapsed when the medication was stopped or reduced,and then used hormones combined with immunosuppressive agents,of which 2 patients did not relapse.Two patients were initially treated with hormones combined with immunosuppressive agents,their condition was well controlled,and none of them relapsed.One patient refused hormone therapy.In one case,symptoms improved significantly after initial hormone therapy alone,and follow-up was terminated due to death.Conclusion MPO-ANCA related HCP is a special type of HCP.For elderly patients with chronic headache and cranial nerve damage,when MPO-ANCA is positive,HCP should be taken into consideration.When there is no pathological diagnosis support,enhanced MRI of the head can assist diagnosis.Hormone combined with immunosuppressant therapy has better effect.