IgG4相关性肺疾病

Immunoglobulin G4 related lung diseases

IgG4相关性疾病是一组临床病理综合征,病因尚不清。IgG4相关性肺疾病在组织病理学表现为IgG4阳性浆细胞及淋巴细胞浸润,伴有间质纤维组织增生。在没有系统器官受累的情况下,IgG4相关性肺疾病在影像学上与非特异性间质性肺炎(NSIP)及肺部肿瘤很难鉴别,因此须依靠病理表现作出确定诊断。此类疾病对糖皮质激素治疗反应良好。

Immunoglobulin G4 （IgG4） related diseases are a group of clinicopathological systemic entities, and the etiology remains unclear. IgG4 related lung disease is histopathologically characterized by infiltration of IgG4-positive plasma cells and lymphocytes in lung tissues along with fibrous interstitial hyperplasia. Under the condition that no other systemic organs are involved,it is difficult to differentiate these diseases from non-specific interstitial pneumonia （NSIP） and lung tumors according to the imaging features. Therefore,final diagnosis may depend on pathological manifestations. IgG4 related diseases have good responses to glucocorticoid treatment.