| Objective: To elucidate the clinical manifestation, serology,imaging features,diagnostic methods and the therapy of autoimmune pancreatitis(AIP) for the purpose of the clinical physician recognition.Methods: A retrospective analysis was conducted among the 7 patients with AIP,who had visited the Beijing shijitan hospital affiliated to capital university of medical science from January 2011 to October 2015. Data of general conditions,clinical manifestation, serology, radiological findings, histology, diagnosis, treatment and prognosis was collected for statistically analysis. Autoimmune pancreatitis was diagnosed according to the international consensus diagnostic criteria(ICDC)established at the 2010 Congress of the International Association of Pancreatology(IAP).Results:(1) General conditions: the age of all patients were above 60 years old, the mean age was 73.4±9.6. 6/7 were male,The sex ratio(male to female) was 6.(2) Clinical symptom: the common symptoms in patients with AIP were jaundice(6/7), weight loss(5/7), anorexia(3/7), abdominal distension(2/7), abdominal pain(1/7). other organ involvement were detected in all 7 patients: All 7 patients with sclerosing cholangitis; 3 of 7 patients with lymphadenopathy; 2 with diabetes; 1 with rheumatoid arthritis.(3) Serology: AIP was characterized by elevated serum levels of immunoglobul Ig G4. the levels of leukocyte, Neutrophils, eosinophils, platelet were within the normal range; 1 of 7 patients hemoglobin decreased; ALT, AST, ALP, GGT in 6 of 7patients with AIP were increased(including 5 patients with jaundice patients); 6 of 7patients with high bilirubin(6 with jaundice),especially direct bilirubin; 1 of 7 with elevated amylase(less than 3 times); 4 of 7 with elevated CRP, 4 of 5 with elevated ESR; CA199 were higher in 6 patients; CA125 were normal; only 1 case with elevated CEA slightly; 3 of 5 patients presented high serum immunoglobulin level in Ig G,all the patients who monitored the level of Ig G4 were> 1.35 g / L; 1 of 7 showed ANA positive weakly; 2 of 7 with elevated RF. 6 patients detected LKM, SMA, AMA,PCA were negative; 4 patients detected ENA negative; 3 of ANCA negative.(4) Imaging finding: 2/4 with abdominal ultrasound demonstrated a diffusely or focally enlarged low-echoic pancreas with clear boundary. 5/6 with CT showed focal or diffuse enlargement of the pancreas, depicted as low density, and delayed enhancement and detected extra-pancreatic lesions:the stenosis of lower bile, it showed “a capsule-like rim surrounded the pancreas in 2 patients. 1 patient with CT found the density of pancreas is uniform while pancreas uncinate displayed abnormal signal by MRI. MRCP showed pancreas swelling in all 7 patients, observing both the dilatation of intrahepatic and Upstream bile duct and the stenosis of lower bile, as "rat tail sign". 1 patient with ERCP revealed the stenosis of lower bile was line like, not mentionged the main pancreatic duct. 5 patients with gastroscopy or duodenal endoscopy, duodenal papilla was red or swelling in 3 patients. 3 patients with 12 M EUS had characteristic imaging: diffuse or focal enlargement of the pancreas presented poor echo texture, mixed grid-like hyperechoic,hypoechoic rim,and normal pancreatic duct. 3/4 with abdominal 18F-FDG PET/CT abnormally increased cellular metabolism.(5) histology: Obtained tissue by duodenal biopsy in 3 patients or pancreatic biopsy in 1 showing abundant lymphoplasmacytic infiltration with Ig G4-positive cells and storiform fibrosis.(6) the response to steroid therapy: 6 of 7 patients who treated with steroid therapy was effective in reducing the clinical symptoms, serology and the radiological signs of pancreatic inflammation. Serum CA199 decreased within the normal range(<37 U/m L); 1 patient who monitored the level of Ig G was normal, 2 monitored Ig G4 were decreased obviously, but still above 1.35 g/L. in addition, 1 of 7 accepted pancreaticoduodenectomy misdiagnosed as pancreatic cancer.(7) Prognosis: 5 patients cured. 1 patients relapse and 1 patient died after surgery.Conclusions: The accurate diagnosis of AIP is an compliated process which combined with clinical symptoms, serology,imaging and histopathological feature.The AIP is rare, the early diagnosis is difficult and easy to miss and misdiagnosis due to atypical symptoms. In order to prevent patients from undergoing unnecessary surgery and improve the quality of life, the clinicians should strengthen awareness of AIP. |
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