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Autoimmune pancreatitis (AIP) is an immune - mediated, special type of chronic pancreatitis, which can involve multiple organs. The clinical manifestation of AIP is complex and varied, making the diagnosis and treatment challenging. With reference to the latest guidelines and studies from both domestic and international sources, this guideline comprises 20 recommendations regarding the diagnosis, treatment, follow-up, and prognosis of AIP. The aim of this guideline is to promote the care capability and improve the outcome of patients with AIP in China. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is a rare disease, and its diagnosis should be made based on a comprehensive evaluation of clinical, radiological, serological, and pathological findings. At present, AIP is classified into two subtypes of type 1 (identified as the pancreatic manifestation of IgG4-related disease) and type 2 (identified as the pancreas-specific disorder independent of IgG4). Although type 1 and type 2 AIP seem to have different pathogeneses, they tend to have similar radiological findings and exhibit a good response to corticosteroid therapy. This article mainly reviews the histopathological features of the two subtypes of AIP, especially the diagnostic challenges encountered in the interpretation of specimens obtained through endoscopic ultrasound-guided fine needle aspiration/biopsy, to as to help pathologists enhance the accuracy of the diagnosis of AIP. [ABSTRACT FROM AUTHOR]

ObjectiveTo evaluate the accuracy of contrast-enhanced ultrasound diagnostic reports for pancreatic lesions.MethodsIn this retrospective study, we included patients who underwent contrast-enhanced ultrasound examination of pancreatic lesions at Peking Union Medical College Hospital from January 2017 to December 2022 and received a confirmed pathological diagnosis. Using pathological diagnosis as the gold standard, the study evaluated the accuracy of contrast-enhanced diagnostic ultrasound. It also analyzed the misdiagnosis of contrast-enhanced ultrasound in diagnosing various pathological types of pancreatic lesions.ResultsOf the 859 patients who met inclusion and exclusion criteria, 489 were male(56.9%) and 370 were female(43.1%).Their age ranged from 16 to 85 years, with a median age of 60(53, 66) years. Except for 47 pancreatic space-occupying lesions that were difficult to categorize as either benign or malignant, a total of 812 cases were included in the diagnostic efficacy analysis of benign and malignant lesions. The results suggested that the diagnostic sensitivity and specificity of contrast-enhanced ultrasound for pancreatic malignant lesions were 98.3%(95% CI: 97.1%-99.1%) and 79.0%(95% CI: 70.1%-86.4%), AUC was 0.887(95% CI: 0.863-0.908), positive and negative predictive value were 96.9%(95% CI: 95.6%-97.9%) and 87.4%(95% CI: 79.6%-92.4%), positive and negative likelihood ratio were 4.69(95% CI: 3.24-6.80) and 0.02(95% CI: 0.01-0.04). The diagnostic accuracy rate of contrast-enhanced ultrasound for diagnosing benign and malignant pancreatic lesions was 95.8%. Of the 859 lesions examined, 48 cases were misdiagnosed by contrast-enhanced ultrasound, with a misdiagnosis rate of 5.6%(48/859), including 7 cases(0.8%) of undetermined diagnosis, while the diagnostic accuracy of pancreatic ductal adenocarcinoma was as high as 98.8%. No complications occurred in any of the patients.ConclusionsContrast-enhanced ultrasound is a safe and effective imaging method for evaluating microvascular perfusion in various pancreatic lesions. It has significant clinical value in diagnosing both benign and malignant pancreatic lesions, particularly in diagnosing pancreatic ductal adenocarcinoma.

Objective To analyze the computed tomography (CT) and magnetic resonance imaging (MRI) findings of immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), to improve the understanding of the imaging features of this disease, and to reduce imaging misdiagnosis. Methods A retrospective analysis was performed on the clinical data of 47 patients with IgG4-related AIP, among whom 33 underwent plain CT scan and contrast-enhanced CT scan, 24 underwent plain MRI scan, contrast-enhanced MRI scan, and magnetic resonance cholangiopancreatography (MRCP) examination, and 10 underwent combined CT and MRI examination to observe the morphology, changes in density/signal, contrast enhancement characteristics of the diseased pancreas, the peripancreatic and pancreatic duct changes, and the manifestations out of the pancreas. Results The CT and MRI scans showed that among the 47 patients, 27 had a “sausage-like”, diffusely enlarged pancreas and 20 had a focally enlarged pancreas. The plain CT scan showed a uniform and slightly low density; the contrast-enhanced CT scan of the lesion showed an inhomogeneous patchy enhancement in arterial phase, and a uniformly delayed progressive enhancement in portal vein phase and delayed phase. The MRI scan of the lesion showed inhomogeneous and slight hypointensity on T1WI-FS and slight hyperintensity on T2WI, the contrast-enhanced MRI scan showed an inhomogeneous mild enhancement in the early stage and a uniformly delayed progressive enhancement on delayed scan. The CT and MRI scans showed that 28 patients had capsule-like edges around the panc-reas, namely “capsule sign”, with a mild to moderate delayed enhancement on contrast-enhanced images. The MRCP showed that 10 patients had segmental stenosis of the main pancreatic duct and localized mild dilatation of the upstream pancreatic duct. The CT and MRI scans showed that 30 patients had changes in the bile duct system, with wall thickening, stenosis, and delayed enrichment of the pancreatic segment of common bile duct. The MRCP showed “beak-like” changes, and dilatation of upstream intrahepatic and extrahepatic bile ducts to varying degrees. Conclusion The CT and MRI findings of IgG4-related AIP have certain characteristics. The combination of imaging findings and relevant laboratory tests can help improve the diagnosis of this disease.

Liver biopsy is a crucial diagnostic tool for chronic liver diseases, allowing for direct observation of lesion morphology and aiding in precise evaluation. It is especially valuable in cases of autoimmune hepatitis, which exhibits complex morphological characteristics. This includes assessing grading and staging criteria, recognizing overlap syndrome, and considering the morphological features of immune-mediated hepatitis and post-COVID-19 vaccination autoimmune hepatitis. Furthermore, significant morphological differences exist between type 1 and type 2 autoimmune pancreatitis, necessitating further research into type 3 autoimmune pancreatitis. [ABSTRACT FROM AUTHOR]

In this paper, we report an elderly patient who had been experiencing poor appetite for one month and yellowing of the skin and sclera for two months before visiting the Department of Geriatrics at Peking Union Medical College Hospital. The patient's blood tests revealed significantly elevated levels of serum total bilirubin, direct bilirubin, and blood sugar. The serum IgG4 level nearly tripled. Abdominal imaging examinations revealed diffuse pancreatic lesions, narrowing of the intrapancreatic segment of the common bile duct, and thickening and marked enhancement of the common hepatic duct, left and right hepatic ducts, and branches. Blood glucose was also significantly elevated during the course of the disease. Moreover, nutritional assessment found that the patient was malnourished. After multidisciplinary discussion, a medium dose of oral methylprednisolone was prescribed to the patient due to his advanced age and multiple chronic conditions. The dose was then quickly reduced while the patient simultaneously received low-dose mycophenolate mofetil (MMF) treatment. As a result, the patient's bilirubin level significantly decreased, jaundice subsided, and his body weight returned to normal with improved physical function. Throughout the diagnosis and treatment process, a multidisciplinary team actively participated in discussions and gradually identified the underlying cause. This exemplifies the crucial role of interdisciplinary collaboration in effectively diagnosing and treating complex illnesses in elderly patients.

Type Ⅰ autoimmune pancreatitis (AIP) is a type of pancreatitis with a predominantly inflammatory and fibrotic nature and is an IgG4-related disease. The pathogenesis of type Ⅰ AIP is still poorly understood, but it is generally believed that it is the result of a combination of genetic, environmental, and immune factors. In recent years, many advances have been made in the cellular and molecular mechanisms of the pathogenesis of type Ⅰ AIP. Therefore, this article aims to review the research progress of the pathogenesis of this disease from the immunological perspective.

Pancreatic sinistral portal hypertension (PSPH) is a rare entity resulting from splenic vein obstruction and is characterized by splenomegaly, gastric and/or esophageal varices with normal liver function. PSPH-related gastric varices is one of severe complications with high incidence and is difficult for prompt diagnosis and treatment. To date, there is no consensus on treatment strategy. Splenic artery embolization (SAE) has been considered as an alternative strategy to splenectomy with satisfactory safety and efficacy for treatment of gastric bleeding caused by PSPH. Here we reported one case with autoimmune pancreatitis-related PSPH with gastric bleeding in Department of Gastroenterology Zhongshan Hospital. Treatment with transcatheter SAE led to gradual stop of gastric bleeding and much improvement of gastric varices. Follow-up study indicated that recurrence of upper gastrointestinal bleeding was not present without postoperative complication. The results suggest that SAE is a minimally invasive, safe and effective treatment strategy for controlling PSPH-related gastric variceal bleeding in the patient with autoimmune pancreatitis.

Objective To investigate the clinical features, diagnosis, treatment, and prognosis of autoimmune pancreatitis ( AIP) alone versus AIP with IgG4 sclerosing cholangitis ( IgG4 - SC). Methods A retrospective analysis was performed for the clinical data of 40 patients with type 1 AIP who were admitted to The First Affiliated Hospital of Zhengzhou University from June 2015 to January 2020, among whom 29 patients had AIP alone and 11 had AIP with IgG4 - SC. The two groups were compared in terms of clinical manifestations, laboratory examination, imaging findings, treatment, and prognosis. The t -test was used for comparison of normally distributed continuous data between two groups, and the Mann - Whitney U test was used for comparison of non - normally distributed continuous data between two groups; the Fisher's exact test was used for comparison of categorical data between two groups. The Kaplan - Meier method was used to calculate recurrence rate and plot recurrence curve, and the log - rank test was used for univariate analysis. Results Compared with the AIP group, the AIP + IgG4 - SC group had significantly higher number of affected organs [ 3 . 0 ( 3. 0 - 4. 0) vs 3. 0 ( 1. 5 - 3. 5 ), Z = - 2. 172, P = 0. 035] and response index before treatment [ 12. 0( 12. 0 - 15. 0) vs 12. 0(9. 0 - 13. 5), Z = - 2. 157, P = 0. 032] . The AIP + IgG4 - SC group had a significantly higher median serum IgG level than the AIP group [ 21. 0 ( 15. 8 - 23 . 7 ) g/ L vs 14. 8 ( 13. 3 - 15. 7 ) g/ L, Z = -2. 711, P = 0. 004]. During the median follow- up time of 15. 8 ( 6. 5 - 3 1. 3) months, the AIP + IgG4 - SC group had a significantly higher recurrence rate than the AIP group (x2 = 8. 155, P = 0. 004 ) . Conclusion Patients with AIP and IgG4 - SC tend to have higher serum IgG4 level, number of affected organs, and recurrence rate than those with AIP alone. Early identification, diagnosis, and treatment can reduce the recurrence rate of AIP. [ABSTRACT FROM AUTHOR]

Objective To summarize the clinical features of autoimmune pancreatitis (AIP) with diabetes as the initial symptom. Methods The clinical data of one case of AIP with diabetes as the initial symptom were analyzed. The 60-year-old male patient was diagnosed in March 2015 in the Department of Endocrinology in China⁃Japan Friendship Hospital. A follow-up study focused on the pancreatic endocrine and exocrine functions was conducted. Results Based on observation, the insulin secretion function in the patient was poor, whereas the glutamic acid decarboxylase antibody, insulin autoantibody and islet cell autoantibody were found negative. In addition, the lipase level was 801 U/L. The swelling of pancreas was found under magnetic resonance cholangiopancreatography. The patients was treated with prednisone for 4 months, the pancreatic swelling ameliorated and the serum lipase level restored to normal. However, the dosage of insulin treatment was unchanged during the prednisone treatment. Conclusions The IgG4 ⁃ related diseases should be paid more attention in suspected diabetes cases, especially in those with clinical characteristics between type 1 and type 2 diabetes, with negative autoantibody and without family history of diabetes, or those with other systematic symptoms. [ABSTRACT FROM AUTHOR]

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IgG4 - related diseases are a class of chronic, progressive, systemic inflammatory disorders that are characterized by lymphoplas-macytic inflammation, as well as elevated IgG4 levels in serum and tissue, and may involve multiple organs or tissues. When involving the pancreas, liver, and biliary tracts, they are called IgG4 - related hepatobiliary diseases. IgG4 - related autoimmune pancreatitis, IgG4 - re-lated sclerosing cholangitis, and IgG4 - associated autoimmune hepatitis are reviewed in terms of their clinical manifestations and laboratory findings. Accurate identification of the laboratory results for IgG4 - related hepatobiliary diseases and correct diagnosis of these diseases help to avoid unnecessary surgery and wrong treatment. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis(AIP) is a special type of pancreatitis, and is because of autoimmune mechanism. Recently published International Consensus Diagnostic Criteria for Autoimmune Pancreatitis include Guidelines of the International Association of Pancreatology, classifying AIP into types 1 and 2, using five cardinal features of AIP, namely imaging of pancreatic parenchyma and duct, serology, other organ involvement, pancreatic histology, and an optional criterion of response to steroid therapy. Extrapancreatic presentations can include sclerosing cholangitis, retroperitoneal fibrosis, sclerosing sialadenitis, lymphadenopathy, nephritis, and interstitial pneumonia. Extrapancreatic lesions could be synchronously or metachronously diagnosed with AIP, sharing the same pathological conditions, showing also a favorable result to corticosteroid therapy and distinct differentiation between Ig G4-related diseases from the inherent lesions of the corresponding organs. In this article we will review this area mainly based on data from abroad. [ABSTRACT FROM AUTHOR]

To analyze and summarize the contrast-enhanced ultrasound (CEUS) characteristics in patients with IgG4 related autoimmune pancreatitis (IgG4-AIP). A total of 54 patients (aged 26 to 76 years old) with IgG4-AIP were collected and retrospectively analyzed. The results demonstrated that there is a variety of enhanced performance on CEUS in patients with IgG4-AIP in the early stage, for some them, especially for those with mass-type pancreatitis, their sonographic appearance and the CEUS characteristics are similar when compared with the patients with pancreatic carcinoma, with varying degrees of low enhancement in the arterial phase, venous phase and delayed stage. Therefore, it is not advisable to make a differential diagnosis based on CEUS alone, and comprehensive evaluation is warranted.

Objective: To summarize the clinicopathological characteristics, diagnosis and treatment of IgG4-related disease (IgG4-RD)., Methods: The clinical data of 43 cases with IgG4-RD diagnosed from January 2013 to December 2017 were retrospectively analyzed. The clinical data of the patients including clinical characteristics, accessory examinations, diagnosis, and treatment were collected., Results: Among the 43 patients with IgG4-RD, the ratio of male to female was 3∶1, the mean age was (51.3±15.9) years. Eleven patients had gastrointestinal symptoms, including 5 cases of IgG4-related cholangitis with the feature of dilation of the biliary system and narrowing of the lumen in the abdominal enhanced CT scans, and 6 cases of IgG4-related autoimmune pancreatitis with the feature of pancreatic enlargement or soft tissue density shadow in the abdominal enhanced CT scans. There were 10 cases (23.3%) with periorbital involvement, with the feature of intraorbital soft tissue nodule in the CT scan. Besides, 9 cases (20.9%) had lymphadenopathy, 6 cases (14.0%) had fever. The results of autoimmune antibody tests showed that 14 of 42 patients had increased antinuclear antibody (ANA). Among 40 patients who underwent immunoglobulin tests, 25 cases had elevated IgG, 12 cases had increased IgA, and 29 cases had increased IgE. Coombs test were performed for 6 cases and 4 patients were positive. Serum immunoglobulin G4 subtypes showed that the IgG4 levels of 35 patients were higher than 1 350 mg/L. The immunohistochemistry showed that IgG4 (+) cells (3->500/HPF) were infiltrated, with the CD20 (+), CD3ε (+) or CD138 (+). Among the 43 patients, 5 patients underwent operations due to misdiagnosis. All patients were treated with steroid and immunosuppressive agents after diagnosis, and their clinical symptoms were improved., Conclusion: The clinical symptoms of IgG4-RD are various, involving multiple organs. Therefore, the standardized diagnosis and treatment of IgG4-RD should be strengthened., (Copyright© by Editorial Board of Journal of Sichuan University (Medical Science Edition).)

Autoimmune pancreatitis (AIP) is a unique subtype of chronic pancreatitis, which shares many clinical presentations with pancreatic ductal adenocarcinoma (PDA). The misdiagnosis of AIP often leads to unnecessary pancreatic resection. 18 F-FDG positron emission tomography/ computed tomography (PET/CT) could provide comprehensive information on the morphology, density, and functional metabolism of the pancreas at the same time. It has been proved to be a promising modality for noninvasive differentiation between AIP and PDA. However, there is a lack of clinical analysis of PET/CT image texture features. Difficulty still remains in differentiating AIP and PDA based on commonly used diagnostic methods. Therefore, this paper studied the differentiation of AIP and PDA based on multi-modality texture features. We utilized multiple feature extraction algorithms to extract the texture features from CT and PET images at first. Then, the Fisher criterion and sequence forward floating selection algorithm (SFFS) combined with support vector machine (SVM) was employed to select the optimal multi-modality feature subset. Finally, the SVM classifier was used to differentiate AIP from PDA. The results prove that texture analysis of lesions helps to achieve accurate differentiation of AIP and PDA.