IgG4相关性肺疾病19例临床分析

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...

Clinical analysis of 19 cases of IgG4-related lung disease

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.