Research progress of clinical and Thoracic Imaging characteristics of IgG4-Related chest Disease
10.3969/j.issn.1006-5725.2024.21.007
- VernacularTitle:IgG4相关疾病临床特征及胸部受累影像学特征研究进展
- Author:
Yifei NI
1
;
Jianping WANG
;
Min LIU
Author Information
1. 中国医学科学院北京协和医学院中日友好临床医学院(北京 100029)
- Keywords:
IgG4-related disease;
chest;
computed tomography
- From:
The Journal of Practical Medicine
2024;40(21):3001-3005
- CountryChina
- Language:Chinese
-
Abstract:
IgG4-related disease(IgG4-RD)is a chronic fibroinflammatory disorder with systemic involve-ment,predominantly affecting middle-aged and elderly males.Although the precise etiology and pathogenesis of the disease remain incompletely understood,its hallmark histopathological features include dense lymphoplasmacytic infiltration with IgG4-positive plasma cells,storiform fibrosis,obliterative phlebitis,and often increased numbers of eosinophils.While more than half of patients with IgG4-RD exhibit elevated serum IgG4 concentrations,this finding lacks specificity as it can also be observed in other conditions;furthermore,a subset of patients may present with normal serum IgG4 levels.Therefore,serum IgG4 elevation is not considered a definitive diagnostic criterion for this condition.The disease is characterized by its broad organ involvement frequently affecting the lymph nodes,salivary glands,lungs among others.The clinical and radiological manifestations of IgG4-RD exhibit a wide range of diver-sity,varying according to the specific organ involved.However,it generally demonstrates a favorable response to corticosteroid therapy while displaying a high propensity for relapse.Thoracic involvement in IgG4-RD presents an array of imaging findings encompassing abnormalities in the lungs,lymph nodes,pleura,chest wall,mediastinum,as well as large vessels such as the aorta and coronary arteries.Accurately differentiating IgG4-RD from malignancies,inflammatory conditions,and other autoimmune diseases poses a significant diagnostic challenge specifically for radi-ologists.This review aims to provide comprehensive insights into the pathophysiology,clinical characteristics,and thoracic imaging features of IgG4-RD with the ultimate goal of facilitating precise diagnosis and thorough evaluation of thoracic involvement.
