抗MPOANCA抗体诊断肥厚性脑膜炎的
摘要：该研究旨在阐明髓过氧化物酶（MPO）抗中性粒细胞胞浆抗体（ANCA）相关的肥厚性硬脑膜炎的特点，发病机理以及治疗策略。回顾性分析36例免疫介导或特发性肥厚性硬脑膜炎患者的临床表现、影像、免疫和病理资料，其中17例MPO-ANCA阳性者，PR3-ANCA阳性者，6例免疫异常者，9例“特发性”患者。MPO-ANCA阳性肥厚性硬脑膜炎的特点是：（一）老年女性居多；（二）根据瓦特法则，82％患者被诊断为肉芽肿与血管炎（常说的韦格纳肉芽肿）；（三）病变局限于硬膜和上呼吸道，表现为进行性头痛，慢性鼻窦炎，中耳炎或乳突炎；（四）少数患者出现局限性或弥漫性肉芽肿血管炎累及上呼吸道、下呼吸道、肾脏；与PR3-ANCA阳性肥厚性硬脑膜炎相比可进展为全身性疾病；（五）根据伯明翰血管炎活动的改良Rankin量表分析，可见轻度神经系统受损；疾病活动度低于PR3-ANCA阳性者。（六）CXCL10、CXCL8、白细胞介素6在脑脊液中含量高，而在尸检或活检硬膜与硬脑膜炎中发现T细胞、嗜中性粒细胞、嗜酸性粒细胞、浆细胞、单核细胞/巨噬细胞数目均升高，提示病变为增生性TH1为主的肉芽肿性硬脑膜炎。（七）强的松和环磷酰胺联合治疗效果高于单用泼尼松。与MPO-ANCA阳性肥厚性硬脑膜炎比较，PR3-ANCA可出现更严重的神经损害，高疾病活动度和高弥散性。然而，PR3-ANCA和MPO-ANCA阳性肥厚性硬脑膜炎患者的免疫学或病理学特征相似，根据瓦特法则，均属于“肉芽肿性血管炎”。这些数据表明，大多数MPO-ANCA阳性肥厚性硬脑膜炎患者应像眼睛、肺、肾脏等局限性血管炎一样，归属于CNS（中枢神经系统）受累的ANCA相关性血管炎。
附原文
-Abstract:Theaimofthisstudywastoelucidatethecharacteristics,pathogenesisandtreatmentstrategyofhypertrophicpachymeningitisthatisassociatedwithmyeloperoxidaseanti-neutrophilcytoplasmicantibody(ANCA).Weretrospectivelyinvestigatedclinical,radiological,immunologicalandpathologicalprofilesof36patientswithimmunemediatedoridiopathichypertrophicpachymeningitis,including17patientswithmyeloperoxidase-ANCA,fourpatientswithproteinase3-ANCA,sixpatientswithotherimmune-mediateddisorders,andninepatientswithidiopathicvariety.Myeloperoxidase-ANCA-positivehypertrophicpachymeningitiswascharacterizedby:(i)anelderlyfemalepredominance;(ii)82%ofpatientsdiagnosedwithgranulomatosiswithpolyangiitis(previouslyknownasWegenersgranulomatosis)accordingtoWattsalgorithm;(iii)ahighfrequencyofpatientswithlesionslimitedtotheduramaterandupperairways,developingheadaches,chronicsinusitis,otitismediaormastoiditis;(iv)alowfrequencyofpatientswiththeclassicalorgeneralizedformofgranulomatosiswithpolyangiitisinvolvingtheentireupperandlowerairwaysandkidney,orprogressingtogeneralizeddisease,incontrasttoproteinase3-ANCA-positivehypertrophicpachymeningitis;(v)lesssevereneurologicaldamageaccordingtothemodifiedRankinScaleandlowdiseaseactivityaccordingtotheBirminghamVasculitisActivityScore鍖椾含鐪嬬櫧鐧滈濂戒笓绉?鍖椾含鐪嬬櫧鐧滈鐤楁晥濂藉尰闄?
