14例累及肺部的IgG4相关性疾病的临床特征  

Clinical characteristics of IgG4-related disease with lung involvement in 14 patients

作　　者：欧彦汝 张莲华 欧阳若芸[1] 梁青春[2] OU Yanru;ZHANG Lianhua;OUYANG Ruoyun;LIANG Qingchun(Department of Respiratory and Critical Care Medicine,Second Xiangya Hospital,Central South University,Changsha 410011;Department of Pathology,Second Xiangya Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院呼吸与危重症医学科,长沙410011 [2]中南大学湘雅二医院病理科,长沙410011

出　　处：《中南大学学报（医学版）》2023年第1期59-67,共9页Journal of Central South University ：Medical Science

基　　金：国家自然科学基金(81970086);湖南省研究生科研创新项目(CX20220363);中南大学中央高校基本科研业务费专项资金(2022ZZTS0281)。

摘　　要：目的:IgG4相关性疾病(immunoglobulin G4-related diseases,IgG4-RD)是一种罕见的自身免疫性疾病,对于肺部受累的患者,目前尚无特异性诊断试验。本研究总结累及肺部的IgG4-RD的临床特征,旨在提高临床医师对该病的认识和诊疗水平。方法:根据2021年《IgG4相关性疾病诊治中国专家共识》中的推荐意见,重新诊断2014年12月至2022年2月中南大学湘雅二医院收治的IgG4-RD患者,分析和总结14例累及肺部的IgG4-RD患者的临床特征。结果:14例患者中,男11例,女3例,确诊中位年龄为66(22~82)岁。6例患者出现咳嗽、咳痰、气促等呼吸系统症状。肺外受累以头颈部腺体多见(6/14),其次为胰腺胆管系统受累(4/14)。患者血清IgG4均升高,11例患者伴有炎症指标异常。肺部影像学表现广泛,纵隔/肺门淋巴结肿大最常见(12/14),其次为双肺多发结节(9/14)、片状密度增高影(7/14)和支气管血管束增多(6/14)。行肺活检者9例,肺外活检者8例。肺活检病理示淋巴浆细胞浸润,5例可见间质纤维化,2例合并静脉炎表现。免疫组织化学结果均可见每高倍镜下阳性IgG4浆细胞>10个,且IgG4/IgG>40%。12例患者给予激素治疗,其中5例为激素联合免疫抑制剂治疗,治疗后10例患者病情缓解,2例病情进展。结论:累及肺部的IgG4-RD较为罕见,临床表现无特异性,肺部影像学多样,诊断应结合血清IgG4水平和病理学检查等进行综合考虑。激素为该疾病的一线治疗药物,视情况联合免疫抑制剂可控制疾病复发。Objective: Immunoglobulin G4-related diseases(IgG4-RD) is a rare autoimmune disease, and there is no specific diagnostic test for patients with lung involvement yet. This study aims to summarize the clinical characteristics of IgG4-RD with lung involvement and improve the understanding and diagnosis of this disease.Methods: All patients diagnosed with IgG4-RD in the Second Xiangya Hospital from December 2014 to February 2022 were re-diagnosed according to the recommendations of Chinese Expert Consensus on the Diagnosis and Treatment of IGG4-Related Diseases in 2021. The clinical data of 14 IgG4-RD patients with pulmonary abnormalities were collected and analyzed.Results: Among the 14 patients, 11 were males and 3 were females, and the median age of diagnosis was 66(22-82) years old. Six cases had respiratory symptoms such as cough, sputum and short breath. Extrapulmonary involvement was the most common in the glands of head and neck(6/14), followed by pancreas and bile duct(4/14). Elevated serum IgG4 level was found in all patients, and most(11/14) were accompanied by abnormal inflammatory markers. Patients’ pulmonary imaging findings were diverse, the most common performances were mediastinal/hilar lymphadenopathy(12/14), followed by multiple pulmonary nodules(9/14), patchy density enhancement(7/14) and the increased broncho vascular bundles(6/14). Lung biopsy was performed in 9 patients, their pathology results showed lymphoplasmic cell infiltration, 5 cases of them had interstitial fibrosis, 2 cases with phlebitis, and extrapulmonary biopsy was performed in 8 patients. Immunohistochemical results of all the patients showed that the number of IgG4+ plasma cells was more than 10 per high magnification, and the ratio of IgG4/IgG was more than 40%. For treatment, 12 patients received hormone therapy, and 5 patients combined immunosuppressive therapy with hormone. 10 patients were in remission after treatment, while 2 patients were progressed.Conclusion: IgG4-RD with lung involvement is rare and has no specific c

关 键 词：IGG4相关性疾病 肺部 临床特征 

分 类 号：R563[医药卫生—呼吸系统] R593.2[医药卫生—内科学]