| Background: Ig G4-related Disease(Ig G4-RD)is a clinicopathological entity systemic disease that is characterized with common histopathological changes and with diverse clinical manifestations and that is often accompanied by elevated serum Ig G4.Since elevated serum Ig G4 can also be found in other diseases,the diagnostic value of serum Ig G4 remains to be further clarified.Ig G4-RD can affect nearly all organs,the pancreas is the most frequently involved organ.Ig G4-related sclerosing cholangitis(Ig G4-SC)is often appeared with Ig G4-Related autoimmune pancreatitis(Ig G4-AIP)simultaneously.Currently the study of Ig G4-SC is not deeply carried out.Ig G4-SC is extremely misdiagnosed owing to its similarity with clinical and imaging findings of biliary and pancreatic malignancies.Since Ig G4-SC responds well to steroid therapy,a definitive diagnosis of Ig G4-SC is very important for reducing misdiagnosis,avoiding delayed treatment and unnecessary surgery.Objective: The aims of this study were to summarize the distribution of patients with different diseases characterized with elevated serum Ig G4 levels(Ig G4> 2.01 g / L)in local region and the clinical features of Ig G4-SC and to assess the diagnostic value of elevated serum Ig G4 level in order to deeply understand Ig G4-SC and to realize the early diagnosis and deferential diagnosis.Method: The patients with elevated serum Ig G4 level and patients who had been diagnosed with Ig G4-RD in the fist hospital of Jilin university from January 1,2012 to December 31,2017 were enrolled in this study.The distribution of different diseases with elevated serum Ig G4 was analyzed.The diagnostic value of serum Ig G4 level in Ig G4-RD was evaluated by statistical analysis.In patients who were diagnosed with Ig G4-SC,the age,sex,initial symptom,serum Ig G4 levels,?-GT,ALP,bilirubin,CA19-9,Ig E,complement and image performance were recorded.The clinical features,biochemical and imaging characteristics of Ig G4–SC were summarized.Additionally,two patients with Ig G4-SC companied either with chronic sclerosing salivary gland inflammation(Kuttner 's tumor)or with autoimmune hepatitis,their clinical and imaging findings,HE and immunohistochemical stainings were described in detail.Result: Of 174 patients with elevated serum Ig G4(Ig G4> 2.01 g / l),110 were Ig G4-RD(63.22%)and 64 were non-Ig G4-RD.Pancreatic,biliary,gallbladder,submandibular gland,parotid gland,lacrimal gland,lymph node,kidney and peritoneum were involved in 110 cases of Ig G4-RD patients.When the serum Ig G4 reached 4.06 g / L(> 2×ULN),the threshold in the diagnosis of Ig G4-RD was obtained with sensitivity of 81% and specificity of 97%.66 cases of Ig G4-SC,male to female ratio was 2.5: 1;initial symptom was jaundice,weight loss was rare.The age distribution of Ig G4-SC was in a range of 30-77 year old,the highest incidence was in a 60-69 year old group.Serum ?-GT,ALP,T-Bil,D-Bil were often increased;Serum Ig G4 was increased significantly in all Ig G4-SC,serum Ig E was elevated in 75% of Ig G4-SC,whereas,serum complement wasn't changed.There were 43 cases(65.15%)with stenosis in the lower part of the common bile duct(CBD),and 11 case(16.67%)with diffusely fibrosis in the intra-and extrahepatic bile ducts.There were only 8 case(12.12%)with stenosis in the bile duct of hepatic portal and in the lower part of the CBD and 4 case(6.06%)with the bile ductal stenosis in hepatic portal.Additionally,one case of Ig G4-SC with Küttner's tumor was admitted originally suspected with hilar cholangiocarcinoma and neck metastasis.The patient's abdominal CT and MRCP showed that the upper common bile duct wall thickening,stenosis was obvious,highly suggestive of cholangiocarcinoma.The patient underwent surgical resection in the left submaxillary region for 1 year.After admission,the patients underwent resection specimens and submandibular gland puncture specimens with HE staining to show lymphocyte plasma-dense infiltration and fibrosis.Immunohistochemical staining showed a large number of Ig G4-positive plasma cells infiltration,the proportion of Ig G4 / Ig G positive plasma cells was greater than 40%.The level of serum Ig G4 increased significantly(38.4g / L).Glucocorticoid steroid treatment effect is good.Another case of Ig G4-SC with autoimmune hepatitis due to hilar mass suspected cholangiocarcinoma underwent left hepatic lobe and cholecystectomy and right hepatic duct and jejunum anastomosis.The patient's abdominal CT and MRCP showed severe hilar bile duct stricture and bile duct wall thickening considering cholangiocarcinoma.HE staining of surgical resection of the bile duct part showed phlebitis and myxoidular fibrosis,and a large number of lymphoplasmacytic infiltration.Immunohistochemical staining showed a large number of Ig G4 positive plasma cells(> 50 / HPF)and Ig G positive plasma cells,Ig G4 / Ig G positive plasma cell ratio(>40%)in resected bile duct sample.Serum Ig G4 levels increased significantly(13.4g / L).Glucocorticoid treatment is effective.Conclusion: Serum Ig G4 elevation can be found in Ig G4-RD and other diseases.Our study indicates that serum Ig G4 level(> 2×ULN)has maximum value in the diagnosis of Ig G4-RD.This study demonstrates that Ig G4-SC does not occur under 30 years old,is rare in the age group of 30~39 years,and is the most common in the age group of 60~69 years.Ig G4-SC alone is only 13.6%,Ig G4-AIP companied with Ig G4-SC are very common(84%).Ig G4-SC manifestations are extremely complex,therefore it's very important to differentiate Ig G4-SC from pancreatic cancer,primary sclerosing cholangitis and cholangiocarcinoma.Clinicians should pay attention to the comprehensive diagnostic value of the clinical symptoms,biochemistry,immune serology,imaginalogy and histology.An early diagnosis should be made to realize reasonable treatment and to avoid unnecessary surgical treatment. |
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