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[抗中性粒细胞胞浆抗体阴性的肉芽肿性多血管炎:一例报告]

[ANCA-negative granulomatosis with polyangiitis: a case report].

作者信息

Sun C H, Li M, Li Q Y, Liang Y, Sun Y C, Zhu X

机构信息

Department of Pulmonary and Critical Care Medicine, Peking University Third Hospital, Beijing 100191, China.

Department of Pathology, Peking University Third Hospital, Beijing 100191, China.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2024 Mar 12;47(3):240-244. doi: 10.3760/cma.j.cn112147-20230813-00073.

DOI:10.3760/cma.j.cn112147-20230813-00073
PMID:38448175
Abstract

We reported a case of 73-year-old male with multiple pulmonary nodules and cavities. The patient was admitted with a chief complaint of "dry cough with shortness of breath for 3 months". Chest CT showed multiple irregular masses, nodules, and patchy lesions in both lungs, accompanied by the formation of cavities. He also had anemia and renal dysfunction. Despite given empirical anti-infective and anti-tuberculosis treatments, the pulmonary nodules progressed, and the cavities enlarged. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative twice. Bronchoscopic biopsy was performed. The mucosal pathology of the right middle lobe lesion showed little necrosis, focal granulomatous structure formation, and relevant vasculitis and remaining vessel wall structure in the necrosis lesions by elastic fiber staining. A clinical diagnosis of ANCA-negative necrotizing granulomatous polyangiitis was made and the patient was treated with glucocorticoids and cyclophosphamide. The nodules and cavities shrank, and some lesions were absorbed.

摘要

我们报告了一例73岁男性,患有多发肺结节和空洞。患者因“干咳伴气短3个月”为主诉入院。胸部CT显示双肺多发不规则肿块、结节及斑片状病变,并伴有空洞形成。患者还存在贫血和肾功能不全。尽管给予了经验性抗感染和抗结核治疗,但肺结节仍进展,空洞增大。抗中性粒细胞胞浆抗体(ANCA)两次检测均为阴性。进行了支气管镜活检。右中叶病变的黏膜病理显示坏死较少,有局灶性肉芽肿结构形成,弹性纤维染色显示坏死病变中有相关血管炎及残留的血管壁结构。临床诊断为ANCA阴性坏死性肉芽肿性多血管炎,患者接受了糖皮质激素和环磷酰胺治疗。结节和空洞缩小,部分病变吸收。

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