呼吸系统IgG4相关性疾病

IgG4‐related disease in respiratory systerm

免疫球蛋白G4相关性疾病(IgG4‐RD)是一种由免疫介导、可在全身不同器官发生的纤维炎性病变,表现为大量IgG4^+浆细胞和淋巴细胞浸润,进而引起脏器肿大、增厚或结节形成,并倾向于形成肿块性损害。IgG4‐RD患者大多伴有血清和组织IgG4水平升高。本文就呼吸系统IgG4‐RD的发病机制、临床表现、诊治和并发症等作一综述。

Immunoglobulin G4‐related disease ( IgG4‐RD ) is an immune‐mesiated fibroinflammatory disorder which can occur in different organs .It is characterized by massive IgG 4^+ plasma cells and lymphocytes infiltration ,which lesds to visceral enlargement , thicking or tubercle formation ,and tends to form mass lesions .Most of the patients with IgG4‐RD are associated with elevated levels of serum and tissue IgG4 .This article reviewed the pathogenesis ,clinical manifestions ,diagnosis , treatment and prognosis of IgG4‐RD in respiratory system .