临床与实验病理学杂志2026，Vol.42Issue(4)：440-445,6.DOI:10.13315/j.cnki.cjcep.2026.04.004
IgG4相关性肺疾病26例临床病理分析
Clinicopathological analysis of 26 cases of IgG4-related lung disease
摘要
Abstract
Objective To improve the understanding of the clinicopathological features of IgG4-related lung dis-ease(IgG4-RLD)and to reduce misdiagnosis and missed diagnosis.Methods Clinicopathological data from 26 pa-tients diagnosed with IgG4-RLD were collected.Morphological and immunohistochemical features were retrospectively analyzed,and relevant literature was reviewed.Results Among the 26 patients,24 were male and 2 were female,with a median age at onset of 62 years.Histologically,all cases showed varying degrees of lymphoplasmacytic infiltra-tion(26/26)and interstitial fibrosis.Obliterative vasculitis was observed in a minority of cases(6/26).Immunopheno-typically,more than 40 IgG4-positive plasma cells per high-power field(HPF)were identified in hotspot areas,and the IgG4/IgG ratio exceeded 40%.Serum IgG4 levels were elevated in 14 cases,within the normal range in 8 cases,and not examined in 4 cases.Conclusion IgG4-RLD is pathologically characterized mainly by lymphoplasmacytic in-filtration and variable degrees of fibrosis,whereas obliterative vasculitis is relatively uncommon.Clinically,the dis-ease predominantly affects middle-aged and elderly men.Serum IgG4 levels have limited diagnostic value and show no significant correlation with patient sex,age,clinical symptoms,lesion consistency,or maximum lesion diameter.关键词
肺部疾病/IgG4相关性疾病/血清IgG4/病理学Key words
lung disease/IgG4-related disease/serum IgG4/pathology分类
医药卫生引用本文复制引用
赵汝楠,张航瑜,胡夏韵,卢艳花,陈鸿远,胡豪,杜明月,何妙侠..IgG4相关性肺疾病26例临床病理分析[J].临床与实验病理学杂志,2026,42(4):440-445,6.基金项目
海军军医大学基础研究面上项目(2024MS011) Basic Research General Program of Naval Medical University(2024MS011) （2024MS011）
