| ObjectiveTo explain the clinical characteristics of the IgG4 related diseases(IgG4-RD),we analyse the clinical cases of IgG4-RD in our hospital.To provide clinical data for clinicians to further understand the disease and attract the attention of clinical workers,so as to make diagnosis and reasonable treatment in the early stage of the disease.MethodsThe clinical data of 147 patients with serum IgG4 examination and IgG4-RD diagnosed in China-Japan Union Hospital of Jilin University from January 2014 to December 2020 were retrospectively analyzed.There were 37 cases in IgG4-RD group and 110 cases in non IgG4-RD group.The general information,first symptoms,signs,first visit department,the course of diagnosis and treatment,laboratory examination,imaging appearance,histopathological and immunohistochemical data of these patients were recorded in detail.The clinical data of 37 patients in IgG4-RD group were statistically analyzed,and the characteristics of the cases were described.The age,gender,past history,allergic history,symptoms and signs,serum IgG4 level,IgG,Ig E,Ig A,Ig M,C3,C4,WBC,Hb,PLT,EO%,EOS,UREA,CREA,fasting blood glucose,AST,ALT,ALP,GGT,PALB,ALB,GLB,TBIL,DBIL,TG,CHOL,Lp(a),erythrocyte sedimentation rate,C-reactive protein,procalcitonin,CA199,AFP,CA125,CEA,CA242,antinuclear antibody were compared between the IgG4-RD group and the non IgG4-RD group;To summarize the characteristics of patients with IgG4-RD,and evaluate the predictive value of serum IgG4 level and IgG4/IgG ratio in the diagnosis of IgG4-RD.Results1.The average onset age of 37 patients in IgG4-RD group was(57.29±14.03)years old,the ratio of men to women was 1.31:1.The common organs involved were pancreas(48.6%),lymph nodes(40.5%)and biliary tract(35.1%).Multiple organ involvement was 83.8% and single organ involvement was only16.2%.Only about a third of the people visited the rheumatology department.2.In IgG4-RD group,patients with allergic history were higher than those in IgG4-RD group,32.4% and 14.5% respectively,and the difference was statistically significant(p=0.017).The serum IgG4 level of IgG4-RD patients in the elevated serum EOS level group was higher than that in the normal and decreased groups,14.56(6.42,18)、 6.13(1.67,6.16)and 5.27(2.26,12.4)respectively,the difference was statistically significant(p=0.036).3.The incidence of diabetes mellitus in IgG4-RD group was higher than that in non IgG4-RD group,11 cases and 10 cases respectively,the difference was statistically significant(p=0.002).The fasting blood glucose in IgG4-RD group was also higher than that in non IgG4-RD group,5.98(5.49,7.76)and5.72(5.05,6.43)respectively,the difference was statistically significant(p=0.037).4.The serum IgG4 level in IgG4-RD group was higher than that in non IgG4-RD group,3.59(2.10,8.94)g/L and 0.53(0.29,1.66)g/L respectively,the difference between the two groups was statistically significant(p<0.001).By drawing ROC curves,the optimal cut-off value of serum IgG4 level in diagnosis of IgG4-RD is 2.01 g/L,the sensitivity is 80.0% and the specificity is 88.3%,p<0.001.The optimal cut-off value of serum IgG4/IgG ratio in diagnosis of IgG4-RD is 0.09,the sensitivity is 94.7% and the specificity is 91.7%,p<0.001.The optimal cut-off value of serum IgG in diagnosis of IgG4-RD is 15.25 g/L,the sensitivity is 73.7%,and the specificity is 77.8%,p<0.001.5.The serum UREA、CREA、AST、GLB level in IgG4-RD group was higher than that in non IgG4-RD group,and the difference was statistically significant(p is 0.001,0.006,0.008,<0.001 respectively).6.After glucocorticoid treatment,most of the patients received clinical remission.One patient had poor curative effect of first treatment with hormone and then relieved with rituximab.Conclusion1.IgG4-RD mainly occurs in middle-aged and elderly patients,most often involving the pancreas,biliary tract,lymph nodes.Mostly of them usually present as multiple organ involvement.However,only a small number of patients visited the Department of Rheumatology Immunology.2.IgG4-RD patients have a history of allergies,often complicated with a history of diabetes.Elevated serum EOS levels contribute to diagnosis.3.IgG4-RD patients are mostly accompanied by increased serum IgG4 level.When the cut-off value of IgG4 is 2.01 g/L,the sensitivity and specificity of diagnosis are the highest,and the serum IgG4/IgG ratio and serum IgG level have predictive value for the diagnosis of the disease.But the serum IgG4/IgG ratio has higher predictive value for the diagnosis of the disease.4.IgG4-RD susceptible to liver and kidney dysfunction.5.Most of patients with IgG4-RD respond well to glucocorticoid therapy,however,biological agents are available for patients with refractory or recurrent. |
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