IgG4相关性肺疾病19例临床分析

Clinical analysis of 19 cases of IgG4-related lung disease

目的通过分析19例IgG4相关性肺疾病(IgG4-RLD)的临床特点,为临床医生对该病的早期诊治提供参考。方法纳入2014年4月至2019年12月首次于四川大学华西医院就诊的19例IgG4-RLD患者作为研究对象,收集并分析其基本临床特征、实验室检查、影像学检查、组织病理学检查、治疗与随访结果等资料。结果19例患者男女比例1.71∶1.00,发病年龄24~74岁,中位年龄56岁。19例患者中15例(78.95%)因一种或多种呼吸道相关症状就诊,其中咳嗽咳痰14例(93.33%),胸痛5例(33.33%),咯血4例(26.67%),气促3例(20.00%),呼吸困难1例(6.67%);因呼吸道外相关症状就诊者4例(21.05%),分别为关节疼痛、腹痛、双下肢水肿、左颈部肿块各1例。10例(52.63%)合并多器官受累,受累部位最多达6个。19例IgG4-RLD患者血清IgG、IgG4水平均升高。CT检查结果显示,实性结节型15例(78.95%),肺泡间质型1例(5.26%),圆形磨玻璃影型1例(5.26%),实性结节型合并肺泡间质型2例(10.53%)。19例患者病理检查均存在不同程度淋巴细胞与浆细胞浸润,少数存在中性粒细胞渗出,淋巴细胞巢或浆细胞巢周围存在特征性纤维化,IgG4阳性浆细胞浸润10~100个/HP不等。单用糖皮质激素治疗8例(42.11%),其中2例(25.00%)复发;糖皮质激素联合免疫抑制剂治疗4例(21.05%);误诊4例(21.05%);出院后确诊3例(15.79%)。结论IgG4-RLD临床表现不特异,容易误诊、漏诊,确诊需借助实验室检查、影像学检查、病理学检查等综合判断,对于肺部占位、肺炎患者应常规筛查血清IgG4水平。

Objective To provide references for clinicians in early diagnosis and treatment of the disease by analyzing the clinical characteristics of 19 cases of IgG4-related lung disease(IgG4-RLD).Methods A total of 19 IgG4-RLD patients who were first treated at the West China Hospital of Sichuan University from April 2014 to December 2019 were included.The basic clinical characteristics,laboratory examination,imaging examination,histopathology examination,treatment and follow-up results were collected and analyzed.Results Of the 19 patients,the male to female ratio was 1.71∶1.00.The age range of onset was 24-74 year-old,and the median age was 56 year-old.15 patients were treated for one or more respiratory-related symptoms(78.95%),of which 14 patients had cough and expectoration(93.33%),and 5 cases had chest pain(33.33%),4 cases had hemoptysis(26.67%),3 cases had tight breath(20.00%)and 1 case had dyspnea(6.67%).4 patients(21.05%)were diagnosed due to extra-respiratory symptoms,including 1 case of joint pain,1 case of abdominal pain,1 case of lower extremity edema,and 1 case of left neck mass.10 patients had multiple organ involvement(52.63%),with a maximum of 6 sites involved.The serum levels of IgG and IgG4 in 19 IgG4-RLD patients were elevated.CT examination showed 15 cases of solid nodular type(78.95%),1 case of alveolar interstitial type(5.26%),1 case of round ground glass shadow type(5.26%),solid nodular type with alveolar interstitial type 2 cases(10.53%).Pathological examination of 19 patients showed various degrees of infiltration of lymphocytes and plasma cells,a small number of neutrophil exudation,characteristic fibrosis around lymphocyte nests or plasma cell nests,IgG4 positive plasma cell infiltration ranging from 10 to 100/HP.8 patients(42.11%)treated with glucocorticoids alone,2 of whom relapsed(25.00%).4 patients(21.05%)treated with glucocorticoids combined with immunosuppressants.4 patients(21.05%)misdiagnosed and 3 cases were confirmed after discharge(15.79%).Conclusion The clinical manifestations of IgG4-RLD are not specific,and it is easy to be misdiagnosed and missed.The diagnosis requires the comprehensive judgment of laboratory tests,imaging tests,and pathological results.Patients with lung occupying and pneumonia should be routinely screened for serum IgG4 level.