累及肺部的肉芽肿性多血管炎1例并文献复习  

Granulomatous polyangiitis involving the lung:a case report and literature review

作　　者：冉雪梅[1] 陈姗姗 邱菊 王开金[1] Ran Xuemei;Chen Shanshan;Qiu Ju;Wang Kaijin(Department of Respiratory and Critical Medicine,Bishan Hospital affiliated to Chongqing Medical University,Chongqing;Department of Internal Medicine,the Second People’s Hospital of Jiangjin District,Chongqing;Department of Respiratory and Critical Medicine,Jiangjin District Central Hospital,Chongqing 402260,China)

机构地区：[1]重庆医科大学附属璧山医院呼吸与危重症医学科 [2]重庆市江津区第二人民医院内科 [3]重庆市江津区中心医院呼吸与危重症医学科,重庆402260

出　　处：《中国组织化学与细胞化学杂志》2022年第5期498-501,507,共5页Chinese Journal of Histochemistry and Cytochemistry

基　　金：重庆市璧山区科技局项目(BSKJ20210034)。

摘　　要：目的探讨肉芽肿性多血管炎(granulomatous polyangiitis,GPA)的临床病理学特征及诊断要点。方法收集重庆市江津区中心医院病理科2020年5月诊断的1例GPA的临床和影像资料,免疫荧光染色检测血液抗中性粒细胞核周型抗体(perinuclear anti-neutrophil cytoplasmic antibody,pANCA)和抗中性粒细胞胞质型抗体(cytoplasmic anti-neutrophil cytoplasmic antibody,cANCA)2项,经皮肺穿刺标本进行HE染色和血管内皮特异性标记物免疫组织化学染色。结果患者血液间接免疫荧光染色pANCA阳性,经皮肺穿刺术病理切片HE染色可见坏死性血管炎、肉芽肿性血管炎,见多核巨细胞、上皮样细胞及组织细胞聚集;经皮肺穿刺术病理切片免疫组织化学染色示血管内皮特异性细胞标志物CD31、CD34表达阳性。结论GPA常累及多器官,仅累及肺部的情况罕见。本例仅累及肺部血管炎,p-ANCA阳性,与大多数GPA不同,但在形态学、免疫组织化学符合GPA特点,临床上容易误诊,需注意鉴别。Objective To investigate the clinicopathological characteristics and diagnosis points of granulomatous polyangiitis(GPA).Methods The clinical and imaging data of a case with GPA diagnosed in the Department of Pathology of Chongqing Jiangjin District Central Hospital in May 2020 were collected.Perinuclear anti-neutrophil cytoplasmic antibody(pANCA)and cytoplasmic anti-neutrophil cytoplasmic antibody(cANCA)in blood were detected by indirect immunofluorescence staining,percutaneous lung puncture specimens were stained with HE and immunohistochemical staining for endothelial specific markers.Results The patient’s blood was positive for p-ANCA by indirect immunofluorescence staining.Percutaneous pulmonary puncture showed necrotizing vasculitis and granulomatous vasculitis,and multinucleated giant cells,epithelioid cells and histiocytes were observed.Immunohistochemical staining showed vascular endothelium specific cell markers CD31 and CD34 were positive in pathological sections of percutaneous lung puncture.Conclusion GPA often involves multiple organs,and it is rare that only the lungs are involved.This case only involves pulmonary vasculitis,and shows pANCA positive,which is different from most GPA.However,the morphology and immunohistochemistry of the case are consistent with the characteristics of GPA,and it is easy to be misdiagnosed clinically,therefore attention should be paid to the differential diagnosis.

关 键 词：P-ANCA 肉芽肿性多血管炎 肺 

分 类 号：R365[医药卫生—病理学]