1Case Report Of Autoimmune Pancreatitis And Clinical Review Of The Chinese Reports

Autoimmune pancreatitis (AIP) is defined as a distinct form of chronic pancreatitis characterized clinically by frequent presentation with obstructive jaundice, generally observed in elderly people and characterized by enlargement of the pancreas and narrowing of the main pancreatic duct with an irregular wall on imaging modalities, the presence of auto-antibodies, elevated levels of immunoglobulins, and pathological features of dense lymphoplasmacytic inflammation and fibrosis. The clinical, laboratory, and radiological features respond dramatically to oral steroid therapy. In this study we try to analyze retrospectively the clinical characters of AIP patients who were treated in our hospital and summarize the clinical features of reported Chinese cases.Part1Clincal analysis of1case of autoimmune pancreatitis from Xiangya hospitalObjectiveDiagnose and analyze the clinical feature of AIP from Xiangya hospital.Methods64Cases and pathological sections from January2006to May2011of Xiangya hospital are collected. Pathological sections are read again and IgG4immunostaining is supplemented. Results1case is diagnosed as AIP of64chronic pancreatitis. The patient is characterized clincally by obstructive jaudice and enlargement of the pancreatic head with delayed rim-like enhancement on CT. Its histopathological feature is characterized by periductal lymphoplasmacytic infiltrate associated with storiform fi brosis, and obliterative phlebitis.the IgG4immunostaining result is less than10IgG4-positive plasma cells per high power field.Conclusions1. This AIP corresponds to Mayo Clinic's diagnostic criteria and International consensus diagnostic Criteria.2. This AIP is type I AIP whose histological description is called lymphoplasma-cytic sclerosing pancreatitis.Part2Review on the Chinese AIP cases which were reported in literaturesObjectiveSummarize the clinical features of reprted Chinese AIP cases and the clinical features compared with Japanese and Korean cases.MethodsThe literatures which reported cases meet the criteria of AIP are selected by searching the Chinese databases including CNKI,VIP, Wanfang Data and Pubmed. Then the clinical and histological features are summarized.ResultsFrom January2004to December2011,199AIP cases meet the diagnostic criterion. The male patients are more than females.The mean age at diagnosis is57.4years old. The main symptoms are obstructive jaundice(69.8%), abdominal pain(52.8%), weight loss(34.7%). Serological abnormalities include mainly elevated titers of IgG and IgG4, and elevated serum levels of CA19-9is not infrequent. The characteristic imaging features include a diffuse(61.9%) or focal(38.1%) enlargement of the gland with delayed rim-like enhancement sometimes. On pancreatography, a diffuse or segmental irregular and narrowing pancreatic duct is highly suggestive of AIP. Chinese AIP cases are reported with involvement of other organs such as sclerosing cholangitis sialadenitis(63.8%), lymphadenectasis(23.1%), sialadenitis(8.0%) and no inflammatory bowel disease. Histological features all shows the presentation of LPSP. The misdiagnosis rate is56.3%.The remission rate of patients with AIP who received steroid treatment is97.7%。Compared with AIP cases in Japan and Korea, the symptoms in Chinese AIP cases of jaundice,weight loss and nausea are more frequent. The elevated serological IgG and IgG4are more frequent. The radiological feature of delayed rim-like enhancement are less frequent. The casees with Retroperitoneal fibrosis and renal involvements are rare. Conclusions1. The Chinses cases, generally observed in elderly men, are characterized clinically by frequent obstructive jaundice, high levels of IgG, IgG4frequently and CA19-9sometimes.2.The radiological imaging on CT scan or MRI is characterized with enlargement of the gland with calcification and pseudocyst somentimes.On pancreatography a irregular, narrow pancreatic duct without upstream ductal dilation are highly suggestive of AIP.3.The Histological feature shows changes of lymphoplasmacytic sclerosing pancreatitis meeting type I AIP.4.The misdiagnosis rate is high, and the remission rate of patients who received steroid treatment is high too.5. Features of AIP are fundamentally similar in Japan,Korea and China.