IgG4-related disease:a retrospective analysis of 20 cases

VernacularTitle:IgG4相关性疾病20例临床特征分析
Author: Kunpeng LI ; Jian ZHU ; Wei ZHAO ; Xiaohu DENG ; Jianglin ZHANG ; Feng HUANG
Publication Type:Journal Article
Keywords: Glucocorticoids; Diagnosis; Therapy; IgG4-related disease
From: Chinese Journal of Rheumatology 2012;(12):820-824
CountryChina
Language:Chinese
Abstract: Objective To understand the features of immunoglobulin G4-related diseases and to improve its diagnosis and treatment.Methods The clinical data of 20 cases discharged from our hospital were analyzed retrospectively.Results Twenty cases with IgG4 related diseases were reviewed retrospectively.IgG4-related diseasesis more prevalent in male than in female (14∶6),and the mean age was 58 years.Pancreas and bile ducts were the most commonly affected organs,2 and more than 2 organs were involved in 17 patients.All patients had significantly elevated serum immunoglobulin G4.Histopathological findings included diffuse tissue fibrosis with extensive lymphoplasmacytes infiltration.On immunohistochemical staining,the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40％.One patient deceased and one patient was left untreated,other patients responded well to the treatment.Conclusion There is no characteristic clinical symptoms for IgG4-related diseases,and serum immunoglobulin G4 is the essential diagnostic method in suspected cases.IgG4-related diseases can be well controlled with prompt use of glucocorticosteroid.