【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。更多还原

【Abstract】 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 clinical manifestation. Its pulmonary imaging is diverse. Diagnosis for it should combine with serum IgG4 level and pathological examination. Glucocorticoid is the first line treatment, and combination with immunosuppressant can help prevent disease recurrence.更多还原