| Part 1.Clinical Research.Clinical analysis of IgG4-related diseaseObjective To summarize and analyze the clinical manifestations,laboratory,imaging and pathological characteristics of IgG4-RD.Methods The clinical data(including general information,clinical manifestation,laboratory,imaging,pathological examination results,treatment and prognosis)of 47 IgG4-RD patients in the Affiliated Hospital of Qingdao University from December 2014 to December 2019,were retrospectively analyzed.Results 1.There were 47 IgG4-RD patients in total,including 37 males and 10 females(M: F 3.7:1).The mean age at diagnosis was 59.60(10.57)years,of which 8 cases were complicated with allergic diseases and 5 cases with malignancy.2.More than one organ were involved in 29 patients.The most common involved organs were pancreas(20 cases),then lymph nodes(13 cases),retroperitoneum(12 cases),lacrimal(11 cases)and then,submandibular gland(11 cases).3.Varying degrees of organ swelling and further compression or obstruction were presented in all the 47 patients.The level of serum IgG4 were elevated in 40 patients,with negative autoantibodies and normal tumor markers.19 patients underwent tissue analysis,10 of which presented with infiltration of IgG4+ plasma cells,storiform fibrosis,and increased IgG4+/IgG+ plasma cell ratio.4.Among the 47 patients,3 cases were untreated,7 were treated with glucocorticoids alone,and the remaining were treated combined with immunosuppressive agents.The most common used immunosuppressive agents were cyclophosphamide,leflunomide and cyclosporine.Follow-up showed that 4 patients treated with glucocorticoids alone did not respond or relapsed,while only 3 patients who were given combined treatment did not respond.Conclusion1.IgG4-RD was mostly found in middle-aged and elderly men,it could be complicated with malignancy or allergic diseases.2.Most patients were presented with multiple organ involvement and the most common involved organs were pancreas,then lymph nodes,then retroperitoneum,then lacrimal and salivary glands.3.Most patients were presented with elevated serum IgG4,negative autoantibodies and normal tumor markers.With different organs involved,the clinical and imaging manifestations were complex and diverse,but the pathological manifestations were similar.4.Glucocorticoid and immunosuppressive therapy were usually effective for IgG4-RD.Part 2.Experimental Research.Application of serum IgG2 and IgG4 in diagnosis of IgG4-related diseaseObjective To explore the application and value of serum IgG2 and IgG4 in the diagnosis and differential diagnosis of IgG4-RD,and determine its the optimal diagnostic threshold to provide a basis for the diagnosis of IgG4-RD.Methods Fourteen IgG4-RD patients,80 AID patients(including 20 RA patients,20 SLE patients,12 SS patients,11 DM patients,17 AIH patients)and 40 healthy controls were enrolled,who were from the Affiliated Hospital of Qingdao University from December 2018 to December 2019.Blood samples of the above patients were collected,and the levels of serum IgG2 and IgG4 were detected by ELISA.Results 1.The serum IgG2 level of 14 patients with IgG4-RD was 5.13±2.74g/L.There was no significant difference between IgG4-RD patients and any other group [(3.99±1.12),(4.79±1.63),(5.25±1.32),(4.49±1.19),(5.50±1.60),(3.89±0.79)vs(5.13±2.74),P>0.05].2.The serum IgG4 level of 14 patients with IgG4-RD was 10.21±11.28g/L,which was obviously higher than other groups,and the difference was statistically significant [(0.50±0.32),(0.59±0.39),(0.59±0.28),(0.44±0.33),(0.71±0.55),(0.41±0.26)vs(10.21±11.28),P<0.05].3.The ROC curve showed that,when serum IgG4=2.055g/L,Youden index was the largest,and the sensitivity was 85.7%,the specificity was 100%,the AUC was 0.964.Conclusion 1.Diagnosis of IgG4-RD based on serum IgG2 level was not yet reliable.2.Serum IgG4,as a biomarker for diagnosis of IgG4-RD,had a high sensitivity and specificity.3.By drawing ROC curve,serum IgG4>2.055g/L was recommended as a diagnostic threshold. |
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