Ig G4相关性疾病(Ig G4-RD)是一种免疫介导的炎症反应伴纤维化疾病,可影响多个器官,受累脏器可出现瘤样病变甚至功能衰竭[1]。该病最常累及的器官有胰腺、肝胆系统、唾液腺、泪腺、腹膜后间隙和淋巴结[2]。我院2015年5月收治1例表现为顽固性胸腔积液的患者,在外院反复抗结核治疗无效,经我院临床检查最终诊断为IgG4-RD,现报告如下, Ig G4-related disease (Ig G4-RD) is an immune-mediated inflammatory response with fibrotic disease that affects multiple organs and may cause tumor-like lesions or even failure in the affected organs [1]. The most commonly affected organs are pancreas, hepatobiliary system, salivary gland, lacrimal gland, retroperitoneal space and lymph nodes [2]. In our hospital in May 2015 admitted to a case of refractory pleural effusion in patients with repeated outside the hospital anti-TB treatment is invalid, the clinical examination of our hospital finally diagnosed as IgG4-RD, are as follows,