脑膜IgG4相关性疾病临床病理特征分析

Clinical and pathological features of IgG4-related sclerosing diseases

目的通过1例脑膜IgG4相关性疾病(Ig G4-SD),回顾并分析Ig G4相关性硬化性脑膜炎的临床、病理特点,免疫组化及与脑膜瘤、浆细胞瘤等鉴别诊断的要点。方法复习临床病例资料,采用免疫组化EnVision法检测vimentin、CK、EMA、CD117、S-100、desmin、SMA、IgG4/IgG和Ki-67。结果 CT及MRI显示病变部位脑膜增厚,增强明显强化。血清学Ig G4增高。病理表现为淋巴-浆细胞炎症反应,纤维化和闭塞性静脉炎,病变边界欠清。免疫组化:vimentin(+),EMA、CK、CD117、S-100、desmin和SMA均(-)、IgG4/IgG>40%、IgG4>10个/HPF、Ki-67增殖指数低。病理诊断为IgG4相关性脑膜病变。结论 IgG4相关性脑膜病变是一种与自身免疫相关的疾病,可单发或伴有其他全身性病变。通过免疫组化方法并结合实验室检查可以明确此病的诊断。

Objective To explore the clinical and pathological features,immunophenotypes and differential diagnosis in Ig G4-related sclerosing pachymeningitis.Methods The clinical manifestations,histopathological features,immunohistochemisty,treatment and prognosis were analyzed in the case of Ig G4-related sclerosing pachymeningitis with review of the literatures.Results CT and MRI revealed meningeal thickening and obvious enhancement in lesions.Serum Ig G4 increased.Pathological manifestation included lymphoplasmacytic inflammatory response,fibrosis and obstructive phlebitis.Immunohistochemical staining showed that cells were positive for vimentin and negative for EMA,CK,CD117,S-100,Desmin and SMA;Ig G4/Ig G was 〉40% and Ig G4 positive cells were 〉10/HPF and Ki-67 proliferation index was low.Pathological diagnosis was Ig G4-related sclerosing pachymeningitis.Conclusions Ig G4-related sclerosing pachymeningitis is an autoimmune-associated disease,which may be single or complicated with other systemic diseases.Based on the typical histopathologic features of this entity,the histochemical and immunohistochemical reactions and the increased concentration of Ig G4 in serum,the diagnosis can be confirmed.