Analysis Of Clinical Caracteristics Of IgG4-related Diseases

Object: IgG4-related disease is a rare clinical disease with multiple organs involved.It was first discovered by Japanese scholars,but there is little understanding and related research on this disease in China.Through the analysis of the IgG4-related diseases clinical characteristics,it will deepen the understanding of the disease,and guide the clinical treatment.Methods: From January 2013 to June 2020,58 patients with IgG4-related diseases diagnosed were collected retrospectively in Shengjing Hospital affiliated to China Medical University.Research is about the Diagnosed time,clinical symptoms,imaging findings and pathological features.Results: Among the 58 cases,there were 29 males and 29 females,with an average age of(57.9 ±9.7)years.A total of 14 organs were involved,and the top 5 involved organs were salivary gland(n=39),lacrimal gland(n=37),pleura(n=19),pancreas(n=18)and biliary system(n=17).The first symptoms were submandibular mass,eyelid swelling and so on.Of the 57 patients who received serum IgG4 detection,55 had elevated IgG4 levels,with an average of(1110 ±1090)mg/d L.Of the 58 patients,8 had peripheral blood eosinophilia,25 had complete Ig E test,17 had elevated serum Ig E,and 34 patients had elevated globulin.23 patients had improved pathological examination,The main pathologic findings were atypical lymphoid tissue hyperplasia,infiltrate of plasma cells and lymphocytes,extensive fibrous tissue hyperplasia,and infiltrate of IgG4 positive plasma cells.Pulmonary imaging showed patchy shadow,interstitial changes,pulmonary vascular filling defect,pleural thickening and pleural effusion.Of the 58 patients,51 received treatment,of which 47 were treated with corticosteroids.39 of the58 patients received treatment for reexamination of IgG4 levels,and 35 patients showed decreased IgG4 levels.Among the 58 patients,8 cases had tumors,5 cases had tumors at different sites from the involved organs,and 10 cases recurred after drug reduction or withdrawal.Conclusion: IgG4-related disease is a systemic disease involving salivary glands and lacrimal glands,which is often accompanied by elevated serum IgG4.Some patients may be accompanied by elevated serum eosinophils and Ig E.The main pathological manifestations are atypical lymphoid hyperplasia,plasma cell and lymphocyte infiltration.Special IgG4 staining of histology is helpful for diagnosis.Glucocorticoid is the first choice of treatment but easy to relapse.And the disease is prone to tumor,and the involved organs can be different from the location of the tumor,which deserves the attention of clinicians.