| Background:Ig G4-related Disease(Ig G4-RD)is a chronic systemic disease that is closely related to serum Ig G4.Ig G4-RD is an autoimmune-mediated disease that can affect multiple organ and tissues.Generally,it is characterized by diffuse or focal swelling,mass formation or nodule formation of the involved tissues and organs.Histopathologic manifestations include a large number of Ig G4 positive plasma cells infiltration,occlusive phlebitis and sheet fibrosis in the affected tissues.Serum Ig G4 level is an important index for the diagnosis of Ig G4-RD.However,the increase of serum Ig G4 is not unique to Ig G4-RD.High levels of Ig G4 can also occur in allergic diseases,viral infections,connective tissue diseases,malignant tumors and other diseases.The pancreas is the most commonly involved organ of Ig G4-RD.The pancreatic manifestation of Ig G4-RD is known as Ig G4-related AIP(type 1 AIP).The clinical manifestations and imaging findings of Ig G4-RD,especially Ig G4-related AIP,are not specific.And the rate of missed diagnosis and misdiagnosis is high.Therefore,it is of great significance for the clear diagnosis of Ig G4-RD and Ig G4-related AIP.Objective:In order to strengthen the understanding and diagnosis of Ig G4-RD and Ig G4-related AIP,we summarized the patients characterized with elevated serum Ig G4 levels(Ig G4> 2.01 g / L)and analyzed the characteristics of Ig G4-RD,especially the clinical features of Ig G4-related AIP in this region.Method:The general data,clinical diagnosis,initial symptoms,signs,laboratory examinations,imaging examinations and pathological histology of 197 patients with elevated serum Ig G4 levels(>2.01g/L)who were hospitalized in the First Bethune Hospital of Jilin University from January 01,2011 to August 01,2018 wereretrospectively analyzed.Statistical analysis was performed on 99 patients of Ig G4-RD and 98 of non-Ig G4-RD with elevated Ig G4.The characteristics of 99 patients with Ig G4-RD were described,and the diagnostic value of Ig G4 levels for Ig G4-RD was evaluated.Seventy-eight patients with Ig G4-related AIP were definitely diagnosed.In patients who were diagnosed with Ig G4-related AIP,The age,sex,symptoms and signs,serum Ig G4 level,AST,ALT,γ-GT、ALP,bilirubin,lipase,amylase,Hb,EO%,complement,CA19-9,Ig E,Ig M,Ig A,Ig G,autoantibodies and imaging manifestations were recorded.The clinical characteristics of Ig G4-related AIP were summarized.Result:Of 197 patients with elevated serum Ig G4(Ig G4> 2.01g/L),99 were Ig G4-RD(50.25%)and 98 were non-Ig G4-RD(49.75%).Among the 99 patients with Ig G4-RD,68% had single organ involvement and 32% had multiple organ involvement,all of which were most common with pancreas involvement.There were 74 males and 25 females in Ig G4-RD patients.The male to female ratio was 2.96:1,and the median age of onset was 60(50,66).The Ig G4 levels in 99 patients with Ig G4-RD [10.60(5.14,15.20)] were significantly different from those in 98 patients with non-Ig G4-RD [3.12(2.50,4.40)](P < 0.001).There were also significant differences in total cholesterol,CA19-9,AST,ALT,γ-GT,ALP,ALB,TB,DB,WBC,EO%,Ig M,Ig A,CRP between Ig G4-RD and non Ig G4-RD groups.The correlation analysis between serum Ig G4 and the above indicators showed no significant correlation with Ig G4,which further proved the diagnostic value of serum Ig G4 level for Ig G4-RD.When serum Ig G4 = 4.37g/L,the sensitivity and specificity of differential diagnosis between Ig G4-RD and non-Ig G4-RD were the highest.The proportion of males and females in 78 patients with Ig G4-related AIP was 2.9:1,and the median age of onset was 61.5(51.5,67.0).The most common initial symptoms were jaundice(43/78,55%),followed by abdominal pain(29/78,37%),abdominal distension,abdominal discomfort(23/78,29%)and weight loss(4/78,5%).In addition,there were rare symptoms such as abnormal liver function,abdominal swelling and mandibular swelling.In 78 patients with Ig G4-related AIP,AST,ALT,γ-GT,ALP,bilirubin and CA19-9 were generally higher than normal.59% of patients had decreased Hb,and17% had increased EO%.Some patients may be combined with decreased complement,increased serum Ig E and ANA positive.In 78 patients,69% of the patients had diffuse pancreatic changes,31% had focal pancreatic changes,and pancreatic head changes were most common in focal pancreatic changes(50%).Of the 78 patients,51 had intrahepatic and extrahepatic bile duct dilatation,9 had intrahepatic bile duct dilatation,and 3 had bile duct dilatation.Conclusion:The increase of serum Ig G4 level can be seen in many diseases.The serum Ig G4 level in Ig G4-RD patients was significantly higher than that in non-Ig G4-RD patients.When the threshold value of Ig G4 was 4.37g/L,the sensitivity and specificity of diagnosing Ig G4-RD were the highest.Pancreas is the most common organ involved in Ig G4-RD.AST,ALT,γ-GT,ALP,bilirubin and CA19-9 in patients with Ig G4-related AIP were generally higher than those in normal controls.Patients with Ig G4-related AIP may have lower Hb and higher EO%.Some patients with Ig G4-related AIP may have decreased complement levels,increased serum Ig E levels and positive autoantibodies such as ANA. |
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