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单纯累及肺部的IgG4相关性疾病:一例病例报告并文献复习

Solely lung-involved IgG4-related disease : a case report and review of the literature.

作者信息

Zhang Xiao-Qin, Chen Guo-Ping, Wu Sheng-Chang, Yu Sa, Wang Hong, Chen Xuan-Yi, Ren Zhuo-Chao

机构信息

Department of Respirology, Zhejiang Provincial People's Hospital, Hangzhou 310014, China.

出版信息

Sarcoidosis Vasc Diffuse Lung Dis. 2016 Dec 23;33(4):398-406.

Abstract

By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement. Solely lung-involved IgG4-RD is not only extremely rare but also easily misdiagnosed as tuberculosis, lung cancer, lymphoma and other common pulmonary diseases. Histopathological examination is the key to the diagnosis of the disease. Corticosteroids are the first choice of treatment, and the overall prognosis is good.

摘要

通过分析1例IgG4相关性肺病(IgG4-RLD)的临床资料并复习文献,作者探讨了IgG4-RLD的临床特征。IgG4-RLD是一种罕见疾病,其特征为血清IgG4显著升高以及大量IgG4+浆细胞浸润。该疾病的临床表现无特异性,影像学特征呈多种类型混合。该疾病不仅可累及肺部,还可累及多个器官。单纯累及肺部的IgG4-RD不仅极为罕见,而且容易被误诊为肺结核、肺癌、淋巴瘤等常见肺部疾病。组织病理学检查是该疾病诊断的关键。糖皮质激素是治疗的首选药物,总体预后良好。

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