IgG4相关肺疾病的临床特征

Clinical Features of IgG4-Related Lung Disease

免疫球蛋白(Immunoglobulin,Ig)G4相关疾病是近年来被报道的自身免疫介导的炎性纤维化疾病,可累及多个器官,导致器官肿大、组织破坏甚至功能衰竭。常见血清IgG4水平升高,病理可表现为淋巴细胞浆细胞炎症,席纹状纤维化,闭塞性静脉炎和IgG4阳性浆细胞增多。IgG4相关疾病的肺部受累称为IgG4相关肺疾病,其亚型可分为累及肺实质、气道、胸膜、纵隔和肺血管,临床表现包括咳嗽、气短、胸痛、咯血和发热,常见影像学表现为肺占位、间质性肺炎、胸腔积液、肺门或纵隔淋巴结肿大等。充分认识IgG4相关肺疾病的临床表现、实验室及病理特点有助于避免误诊,改善疾病预后。

IgG4-related disease is a recently described immune mediated fibroinflammatory disease that can affect multiple organs and lead to organomegaly, tissue destructive lesions and organ failure. Serum IgG4 level may elevated and pathological characters are lymphoplasmacytic inflammation, storiform fibrosis, phlebitis and increased numbers of IgG4-positive plasma cells. IgG4-related disease with pulmonary involvement called IgG4-related lung disease, which of the subtypes include involvement of the lung parenchyma, airways, pleura, mediastinum and pulmonary vessels. The clinical symptoms include cough, dyspnea, chest pain, hemoptysis and fever. The common imaging findings were lung occupying, interstitial pneumonia, pleural effusion, enlargement of hilar or mediastinal lymph nodes. Full understanding of the clinical manifestations, laboratory testing and histopathological features of IgG4 related lung diseases may help avoid misdiagnosis and improve the prognosis.