The Department of Clinical Research.
The Department of Respiratory Medicine, National Hospital Organization Yamaguchi Ube Medical Center, Ube, Japan.
Curr Opin Pulm Med. 2019 Jul;25(4):384-390. doi: 10.1097/MCP.0000000000000581.
The causes of exudative pleural effusions are diverse and frequently remain unclear despite exhaustive examinations. Recently recognized IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that can affect nearly any organ including the lungs. This review will focus on the involvement of IgG4 in exudative pleural effusion of unknown cause.
IgG4 is found to be involved in a proportion of patients with undiagnosed pleural effusions. Pleural involvement in IgG4-RD can be seen in isolation or association with other organ disease. Pleural thickening and/or effusion are common clinical features of IgG4-related pleural lesions, and this condition is histologically characterized by a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells in the pleura. Although the pathogenesis of IgG4-RD is poorly understood, there is a growing body of evidence that indicates an antigen-driven process requiring T-cell and B-cell interaction in which autoantibodies, plasmablasts, follicular helper T cells and CD4+ cytotoxic T lymphocytes participate.
The possibility of IgG4-related pleural lesion should be considered in patients with pleural effusion of unexplained cause when lymphoplasmacytic infiltration is seen in a pleural biopsy specimen. This condition is responsive to systemic steroid therapy.
尽管进行了详尽的检查,渗出性胸腔积液的病因仍多种多样且常常不明确。最近认识到的 IgG4 相关疾病(IgG4-RD)是一种纤维炎性疾病,可影响包括肺在内的几乎任何器官。这篇综述将重点介绍 IgG4 在不明原因渗出性胸腔积液中的作用。
已发现一部分原因不明的胸腔积液患者存在 IgG4 参与。IgG4-RD 可累及胸膜,也可与其他器官疾病相关联。胸膜增厚和/或胸腔积液是 IgG4 相关胸膜病变的常见临床特征,这种疾病在组织学上的特征是胸膜中富含 IgG4 阳性浆细胞的淋巴浆细胞浸润。尽管 IgG4-RD 的发病机制尚不清楚,但越来越多的证据表明这是一种抗原驱动的过程,需要 T 细胞和 B 细胞相互作用,其中自身抗体、浆母细胞、滤泡辅助 T 细胞和 CD4+细胞毒性 T 淋巴细胞参与其中。
当胸腔积液活检标本中出现淋巴浆细胞浸润时,对于原因不明的胸腔积液患者,应考虑存在 IgG4 相关胸膜病变的可能性。这种情况对全身类固醇治疗有反应。