IgG4相关性肺疾病临床特征分析

Clinical analysis of IgG4-related lung disease

目的分析IgG4相关性肺疾病（IgG4－RLD）的临床特征。方法回顾性调查2007年12月—2017年12月复旦大学附属中山医院收治并经病理诊断的13例IgG4－RLD患者的病例资料，对其基本临床资料、影像、血清IgG4水平、病理学资料、治疗和预后进行分析，总结其临床特征。结果13例IgG4－RLD患者中男8例，女5例，年龄（51.1±14.8）岁。临床表现多样，10例出现呼吸道症状，主要表现咳嗽9例、咳痰6例，咯血6例，胸闷2例，气喘2例；12例合并多器官受累。胸部CT表现为实性结节团块型9例，圆形磨玻璃型1例，肺泡间质型3例，支气管血管束型3例，以实性结节团块型为主，纵隔及双侧肺门肿大淋巴结10例。13例患者血清IgG4水平均升高，为（7.92±13.98）g/L。受累肺组织具有高度相似的病理改变，表现为大量淋巴细胞、浆细胞浸润且合并纤维化，大量IgG4阳性浆细胞浸润（10～320个/高倍视野），IgG4阳性浆细胞/IgG阳性浆细胞〉40%。单纯糖皮质激素治疗8例，联合免疫抑制剂治疗5例，经治疗后10例病情缓解，3例病情进展，无死亡病例。结论IgG4－RLD的临床及影像学表现多样，病变常可累及多个器官，血清IgG4升高及肺组织病理特征性异常有助于确诊；糖皮质激素可作为初治IgG4－RLD的首选治疗。

ObjectiveTo improve the understanding and treatment of IgG4-related lung disease （IgG4-RLD） by analyzing the clinical characteristics of patients.MethodsA total of 13 patients with IgG4-related lung disease （IgG4-RLD） diagnosed by pathology at Zhongshan Hospital affiliated to Fudan University during December 2007 to December 2017 were included. The clinical characteristics, chest CT, pathological features, serum IgG4 levels, therapy and prognosis of these 13 patients were analyzed.ResultsThe 13 patients with IgG4-RLD included 8 men and 5 women, with an average age of （51.1±14.8） years. The clinical manifestations were varied, 10 patients showed respiratory symptoms, mainly including cough （n=9）, expectoration （n=6）, hemoptysis （n=6）, chest tightness （n=2） and breathless （n=2）. Multiple organs were involved in 12 cases. Chest CT showed solid lung nodules （n=9）, ground glass shadows （n=1）, alveolar-interstitial infiltration （n=3）, bronchovascular lesions （n=3）, and the solid lung nodules was predominant. Enlargement of lymph nodes in the mediastinal and bilateral hilum of the lung were present in 10 cases. Serum IgG4 levels were elevated in all the 13 cases, with an average concentration of （7.92 ±13.98）g/L. Quite amount of lymphocytes, plasma cell infiltration and fibrosis were common pathologic findings. Immunohistochemical staining showed a large number of IgG4 positive plasma cells infiltration with IgG4 positive plasma cells count 10-320/HP in 13 cases. The ratio of IgG4 positive plasma cells to IgG positive plasma cells was higher than 40%. Eight patients received glucocorticoid therapy alone, five received glucocorticoids with immunosuppressant therapy. After treatment, 10 patients were with remission, and disease progressed in 3 patients, but no death.ConclusionsThe clinical manifestations and imaging features of IgG4-RLD are diverse, and lesions can often involve multiple organs. The diagnosis could be made based on pathological features and IgG4 serum levels. Glucocorticoid can be used as the first choice for the treatment of IgG4-RLD.