目的探讨肺玻璃样变性肉芽肿的临床病理特征。方法报道1例肺玻璃样变性肉芽肿病例的临床及病理特点,并复习文献探讨其诊断及鉴别诊断。结果肺玻璃样变性肉芽肿病理学表现由大片玻璃样变性的厚的胶原带组成,呈漩涡状、束状排列,胶原带之间见较多淋巴浆细胞浸润;中心区及边缘区见淋巴滤泡结构。免疫组化CD99、Bcl-2、ALK阴性,IgG4/IgG阳性浆细胞之比>40%。Masson三色染色显示为胶原纤维阳性;刚果红染色阴性。结论病理检查是肺玻璃样变性肉芽肿确诊的关键。其发病机制与免疫反应有关,可能为IgG4相关性疾病谱的一种。 Objective To investigate the clinicopathological features of pulmonary vitreous degeneration granuloma. Methods A case of pulmonary vitreous degeneration granuloma clinical and pathological features reported, and review the literature to explore its diagnosis and differential diagnosis. Results The pathological manifestations of lung-like granulomatous granuloma consisted of a large area of ​​glass-like degeneration of thick collagen bands, arranged in a swirling pattern with many infiltration of lymphocytes in the collagen bands. In the central area and marginal zone, lymphatic drainage Bubble structure. Immunohistochemical CD99, Bcl-2, ALK negative, IgG4 / IgG positive plasma cells ratio> 40%. Masson trichrome staining showed collagen fibers positive; Congo red staining was negative. Conclusions Pathological examination is the key to the diagnosis of pulmonary vitreous granuloma. Its pathogenesis is related to the immune response and may be one of the IgG4-related disease spectrum.