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Radiologists play a key role in establishing an early and accurate diagnosis, especially for rare diseases. Mahvash disease (OMIM 619290) is an autosomal recessive hereditary disease caused by inactivating mutations of the glucagon receptor and its main clinical consequences are pancreatic neuroendocrine tumors and in some cases, porto-sinusoidal vascular disease and portal hypertension. Untreated Mahvash disease can be lethal. The diagnosis of Mahvash disease has almost always been delayed in the past due to radiologists unawareness of or unfamiliarity with the unique imaging features of Mahvash disease which are moderately to enormously enlarge pancreas with preserved pancreas contour and parenchyma without vascular involvement or lymphadenopathy. These features help differentiate Mahvash disease from other etiologies of diffusely enlarged pancreas such as diffuse pancreatic ductal carcinoma, diffuse pancreatic lymphoma, and autoimmune pancreatitis. Invoking Mahvash disease in the differential diagnosis of an enlarged pancreas has recently been shown to facilitate early diagnosis. To prevent missing the diagnosis of this significant disease, I sincerely ask radiologists to consider Mahvash disease in their differential diagnoses of diffusely enlarged pancreas.

Background/Objectives: Autoimmune pancreatitis (AIP) is a diagnosis-challenging disease that often mimics pancreatic malignancy. Pancreatic resection is considered to be a curative treatment for pancreatic ductal adenocarcinoma (PDAC). This meta-analysis aims to study the incidence of AIP in patients who have undergone pancreatic resection for clinical manifestation of cancer. Methods: A comprehensive search was conducted in three databases, PubMed, Embase and the Cochrane Library, using the terms 'autoimmune pancreatitis' and 'pancreatic resection' and supplemented by manual checks of reference lists in all retrieved articles. Results: Ten articles were included in the final analysis. 8917 pancreatic resections were performed because of a clinical suspicion of pancreatic cancer. AIP accounted for 140 cases (1.6%). Type 1 AIP comprised the majority of cases, representing 94% (132 cases), while type 2 AIP made up the remaining 6% (eight cases) after further classification. AIP accounted for almost 26% of all cases of benign diseases involving unnecessary surgery and was overrepresented in males in 70% of cases compared to 30% in females. The mean age for AIP patients was 59 years. Serum CA 19 − 9 levels were elevated in 23 out of 47 (49%) AIP patients, where higher levels were detected more frequently in patients with type 1 AIP (51%, 22 out of 43) than in those with type 2 AIP (25%, 1 out of 4). The sensitivity of IgG4 levels in type 1 AIP was low (43%, 21/49 patients). Conclusion: Even with modern diagnostic methods, distinguishing between AIP and PDAC can still be challenging, thus potentially resulting in unnecessary surgical procedures in some cases. Serum CA 19 − 9 levels are not useful in distinguishing between AIP and PDAC. Work must thus be done to improve diagnostic methods and avoid unnecessary complicated surgery. [ABSTRACT FROM AUTHOR]

Objective Methods Results Conclusions In this study we aimed to comprehensively evaluate the clinical features and treatment outcomes of Chinese patients with autoimmune pancreatitis (AIP) through a single‐center real‐world study.Patients diagnosed with AIP in Changhai Hospital, Naval Medical University from January 2014 to December 2021 were included. Baseline characteristics, laboratory test results, cross‐sectional imaging and endoscopic ultrasound (EUS) findings, and long‐term follow‐up data were obtained. The differences in these characteristics between type 1 and type 2 AIP patients were analyzed.Among all 320 patients, 271 (84.7%) and 49 (15.3%) had type 1 and type 2 AIP, respectively. The most common initial symptom was abdominal discomfort (58.1%), followed by obstructive jaundice (32.5%). Extrapancreatic organ involvement was identified in 126 (39.4%) patients, with the biliary system being the most commonly involved (36.6%). Elevated serum IgG4 level was rare in type 2 AIP patients. The diagnostic accuracy of computed tomography (CT), magnetic resonance imaging (MRI), and EUS for definitive and probable AIP were 78.0%, 68.7%, and 80.5%, respectively. EUS‐guided tissue acquisition with immunohistochemical staining helped establish a final diagnosis in 39.7% of patients. During the follow‐up period of 60 months, 18.6% of patients experienced relapse. The 1‐, 3‐, and 5‐year relapse rates were higher in type 1 AIP patients, with an accumulated rate of 8.0%, 12.6%, and 15.1%, when compared with those with type 2 AIP.Type 2 AIP is not uncommon in Chinese population. The diagnostic accuracy of CT and EUS for AIP might be superior to that of MRI. [ABSTRACT FROM AUTHOR]

Objectives: While autoimmune pancreatitis (AIP) responds well to steroid therapy, the high relapse rate in type 1 AIP remains a critical problem. The present study examined predictors of relapse of type 1 AIP following steroid therapy. Materials and methods: Nine factors potentially predictive of relapse were analyzed in 81 AIP patients receiving steroid therapy with follow-up ≥ 12 months. The rate of serum IgG4 decrease following steroid therapy was calculated by dividing the difference between serum IgG4 values before and at two months after the start of steroid by the IgG4 value before steroid. Results: A relapse occurred in 11 patients (13.5%) during a median of 38 months. Multivariate analysis revealed that the presence of IgG4-related retroperitoneal fibrosis (HR: 5.59; 95% CI: 1.42–22.0; p = 0.014) and the low rate of serum IgG4 decrease after steroid therapy (HR: 0.048; 95% CI: 0.005–0.46; p = 0.008) were significant, independent predictors of AIP relapse. The cut-off value based on receiver operating characteristic curve data for the rate of serum IgG4 decrease before and at two months after steroid therapy distinguishing patients with and without a relapse was 0.65. Using this cut-off value, the area under the curve, sensitivity, and specificity were found to be 0.63, 0.73, and 0.60, respectively. Conclusion: The low rate of serum IgG4 decrease after the start of steroid therapy and the presence of IgG4-related retroperitoneal fibrosis were predictive of type 1 AIP relapse. Cautious, gradual tapering of steroid dosage and longer maintenance therapy are recommended for patients with these factors. [ABSTRACT FROM AUTHOR]

To our best knowledge, the formation of a pancreatico-renal fistula and the presence of pancreatic fluid collection in the renal subcapsular space have not been reported as autoimmune pancreatitis (AIP) complications. We describe a case of a pancreatico-renal fistula associated with type 1 AIP. The patient presented with abdominal and back pain accompanied by pancreatic cystic lesions during an untreated course of AIP. The diagnosis of pancreatico-renal fistula was based on the presence of a left renal subcapsular fluid collection containing pancreatic amylase, disappearance of pancreatic cysts, and a defect in the partial anterior renal fascia observed on imaging studies. Treatment with steroids and percutaneous drainage resulted in improvement. Pancreatic pseudocysts can affect other organs owing to their digestive action. Similar symptoms may occur in patients with AIP. [ABSTRACT FROM AUTHOR]

A 50-year-old woman was referred to our hospital with elevated serum amylase levels. Physical examination revealed no jaundice or abdominal tenderness. Serum IgG4 was negative. Computed tomography revealed a localized pancreatic duct narrowing in the pancreatic head, with caudal pancreatic duct dilation and an intraductal papillary mucinous neoplasm. Pancreatic enlargement was not observed. Endoscopic ultrasonography (EUS) showed a small hypoechoic mass. Although EUS-guided, fine-needle aspiration was performed, no diagnosis was established. Endoscopic retrograde pancreatography showed a localized narrowing in the main pancreatic duct of the pancreatic head. A biopsy of the narrowing was performed through the minor papilla because of difficult access from the major papilla. The specimen showed the infiltration of numerous IgG4-positive plasma cells, suggesting type 1 autoimmune pancreatitis (AIP). Six months later, magnetic resonance cholangiopancreatography revealed improvement in the narrowing without specific treatment. The patient presented with localized narrowing of the pancreatic duct and caudal duct dilation, which was distinct from pancreatic cancer. Diagnostic difficulties arose from negative serum IgG4 results, the lack of typical imaging characteristics of AIP, and failure to meet the AIP criteria according to the relevant Japanese and international guidelines. However, AIP was suspected and surgery was successfully avoided through a biopsy. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is identified as an outlier in the clinical practice of chronic pancreatitis caused by autoimmune system dysfunction. AIP is classified into 3 subtypes: AIP type 1 and AIP type 2, which are both sensitive to corticosteroids, and the recently introduced AIP type 3. Case Report: We present a case of a patient who presented with painless obstructive jaundice. Computed tomography (CT) revealed hyperdense gallbladder material, further dilatation of intrahepatic bile ducts, and distention of the bile duct (15 mm). Based on the available clinical data, which were strongly compatible with pancreatic cancer, Whipple surgery was selected as the treatment for this case. The consequent histopathological report revealed areas of pancreatic parenchyma with fibrous connective tissue development and dense inflammatory cell infiltration with lymphocytes and plasmacytes, which showcased IgG4 positivity. The clinical results suggested a diagnosis of AIP type 1, and the patient was referred to his treating physician for further treatment of AIP. Preoperative histological examination of the pancreas, along with evaluation of the radiological and serological features, could have aided in determining the diagnosis of AIP type 1 pancreatitis despite the unique abnormality of this particular case. Conclusions: Given the aforementioned conditions, AIP, even as a rare clinical entity, emerges as a canonical ailment and should be considered a viable possibility in clinical practice since it can exclude the patient from an unnecessary surgery [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis type 2 is a relatively novel entity with some still controversial issues. The current diagnostic algorithm relies on imaging studies and histology. Therapy includes corticosteroids with consequently low risk of relapse in the following year. However, the pathogenesis remains unclear, and data are insufficient for long-term prognosis. We have treated a 17-year-old boy whose autoimmune pancreatitis type 2 was revealed during surgery for a pre-existing biliary tract anomaly with concurrent protozoal infection. We discuss the co-occurrence of these conditions in terms of eventual pathogenesis correlation and combined effect on long-term prognosis. [ABSTRACT FROM AUTHOR]

Abstract Background/Objectives Autoimmune pancreatitis (AIP) is a diagnosis-challenging disease that often mimics pancreatic malignancy. Pancreatic resection is considered to be a curative treatment for pancreatic ductal adenocarcinoma (PDAC). This meta-analysis aims to study the incidence of AIP in patients who have undergone pancreatic resection for clinical manifestation of cancer. Methods A comprehensive search was conducted in three databases, PubMed, Embase and the Cochrane Library, using the terms ‘autoimmune pancreatitis’ and ‘pancreatic resection’ and supplemented by manual checks of reference lists in all retrieved articles. Results Ten articles were included in the final analysis. 8917 pancreatic resections were performed because of a clinical suspicion of pancreatic cancer. AIP accounted for 140 cases (1.6%). Type 1 AIP comprised the majority of cases, representing 94% (132 cases), while type 2 AIP made up the remaining 6% (eight cases) after further classification. AIP accounted for almost 26% of all cases of benign diseases involving unnecessary surgery and was overrepresented in males in 70% of cases compared to 30% in females. The mean age for AIP patients was 59 years. Serum CA 19 − 9 levels were elevated in 23 out of 47 (49%) AIP patients, where higher levels were detected more frequently in patients with type 1 AIP (51%, 22 out of 43) than in those with type 2 AIP (25%, 1 out of 4). The sensitivity of IgG4 levels in type 1 AIP was low (43%, 21/49 patients). Conclusion Even with modern diagnostic methods, distinguishing between AIP and PDAC can still be challenging, thus potentially resulting in unnecessary surgical procedures in some cases. Serum CA 19 − 9 levels are not useful in distinguishing between AIP and PDAC. Work must thus be done to improve diagnostic methods and avoid unnecessary complicated surgery.

We report a case of a 44-year-old male patient, who presented to the University Hospital of Salzburg, Austria with abdominal pain, persistent jaundice, and lack of appetite. Radiological work-up (CT, MRI, PET/CT) indicated a suspicious mass of the uncinate process of the pancreatic head with adjacent infiltration and regional lymphadenopathy. The differential diagnosis was between primary pancreatic cancer and focal autoimmune pancreatitis. Further laparoscopic biopsies from multiple areas, showed only fibrous scarring processes, with no malignancy. Treatment with steroids didn't give any benefits. After multiple follow-up CTs and MRs within 6 months—additional biopsies were done, which eventually demonstrated adenocarcinoma. Evidently the cancer diagnosis was much delayed and the patient started receiving chemotherapy, but radical surgery was not possible. Multiple articles and case reports can be found in the literature, that are reviewing the fact that pancreatic inflammatory processes are mimicking pancreatic tumor, but not many articles or case reports are available in the literature, where neoplastic processes are misinterpreted as inflammatory and incorrectly proven with histological examination. One of the main reasons for improper diagnosis is the desmoplastic reaction around the pancreatic malignancy. Another important aspect is the acceptance of histological diagnosis as conclusive, where no opposing arguments are specified, based on radiological criteria.

Objectives: This study aims to evaluate the utility of F-18 FDG PET/CT in the non-invasive diagnosis of autoimmune pancreatitis (AIP) and differentiating it from pancreatic cancer (CaP) based on the amount and pattern of FDG uptake, as well as involvement of extra-pancreatic sites. Methods: A systematic search was conducted using PubMed, Scopus, Cochrane Library and Google Scholar. Only those studies that compared the findings of F-18 FDG PET/CT in terms of SUVmax, pattern of FDG uptake and presence of FDG-avid extra-pancreatic sites in both AIP and CaP were included. Studies were qualitatively assessed for risk of bias and publication bias. The diagnostic performance of parameters on PET/CT was examined through pooled sensitivity, specificity, diagnostic odd's ratio (DOR) and summary receiver operator characteristic (SROC) curve analysis. Results: Six studies were included with a total of 580 patients. 178 patients had AIP (Age 18–90 years, male, M: female, F ratio—8.4:1) and 402 patients had CaP (Age 22–88 years, M:F ratio-1.5:1). Type of AIP was reported in only 3 studies, with the included cases predominantly being type 1 AIP. All studies were retrospective with heterogeneity and a risk on patient selection and index test. The FDG uptake, expressed as SUVmax, was lower in AIP with a weighted mean difference of −3.11 (95% confidence interval, CI: −5.28 to −0.94). To diagnose AIP, the pooled sensitivity, specificity and DOR of diffuse pattern of FDG uptake were 0.59 (95% CI: 0.51–0.66), 0.89 (95% CI: 0.86–0.92) and 21.07 (95% CI: 5.07–88.32), respectively, with an area under curve (AUC) of 0.717 on SROC analysis. The pooled sensitivity, specificity and DOR of FDG-avid extra pancreatic sites were 0.55 (95% CI: 0.45–0.65), 0.58 (95% CI: 0.52–0.64) and 2.33 (95% CI: 1.40–3.89), respectively, with an AUC of 0.632. Conclusion: On F-18 FDG PET/CT, a pancreatic lesion of AIP has a lower SUVmax value than CaP. A diffuse pattern of FDG uptake and presence of an extra-pancreatic FDG-avid site are nearly 21 times and twice more likely in AIP than CaP, respectively. [ABSTRACT FROM AUTHOR]

Objectives: Autoimmune pancreatitis (AIP) type 1, paraduodenal (groove) pancreatitis, and follicular pancreatitis are rare clinical entities whose diagnosis may be challenging, given the potential imaging overlap with pancreatic cancer. Methods: We performed a retrospective analysis of the medical chart of a patient with multiple pancreas pathologies. Results: We present a case with multiple pancreas pathologies, including a poorly differentiated ductal adenocarcinoma of pancreatobiliary type, an intraductal papillary mucinous lesion (pre-existing lesion of IPMN type), and an inflammatory process with complex features, in which paraduodenal (groove) pancreatitis, follicular pancreatitis, and IgG4-related pancreatitis (AIP type 1) were also present. Conclusions: The diagnosis of AIP and paraduodenal pancreatitis is not always straightforward, and in some cases, it is not easy to differentiate them from pancreatic cancer. Surgery should be considered in patients when a suspicion of malignant/premalignant lesions cannot be excluded after a complete diagnostic work-up. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis is characterized by infiltration of the pancreas parenchyma by immunoglobulin G4 (IgG4)-positive plasma cells, chronic inflammation, and fibrosis. It can affect the entire body depending on the subtype. Distinguishing mass-forming type autoimmune pancreatitis from pancreatic cancer can often be challenging. A 60-year-old male presented to our outpatient clinic with abdominal discomfort. Computed tomography revealed a 4.8 cm mass in the pancreatic head obstructing the common bile duct. Endoscopic ultrasound-guided fine needle biopsy and endoscopic retrograde cholangiopancreatography with biliary drainage were performed. Pathological examination revealed no malignant cells, but more than 20 IgG4-positive cells were observed. Following two weeks of steroid treatment, the patient's symptoms improved, and follow-up imaging showed a reduction in the size of the pancreatic head mass. Herein, we report a case of mass-forming autoimmune pancreatitis initially misdiagnosed as pancreatic cancer. [ABSTRACT FROM AUTHOR]

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 unique form of chronic pancreatitis with a multifactorial pathogenesis. Historically, it has been classified as type 1 and type 2, according to its clinical and histological features. The diagnosis of AIP is challenging and relies on a combination of clinical, histopathologic, serologic, and imaging characteristics. In the available guidelines, the imaging hallmarks of AIP are based on cross-sectional imaging and cholangiopancreatography retrograde endoscopic findings. Endoscopic ultrasound (EUS) is generally used for pancreatic tissue acquisition to rule out pancreatic cancer and diagnose AIP with limited accuracy. Several papers reported the reliability of EUS for providing informative morphologic features of AIP. Nowadays, the improvement in the resolution of EUS conventional images and the development of new ancillary technologies have further increased the diagnostic yield of EUS: contrast-enhanced EUS and EUS elastography are non-invasive and real-time techniques that strongly support the diagnosis and management of pancreatic diseases. In this review article, we will present the role of conventional EUS and ancillary diagnostic techniques in the diagnosis of AIP to support clinicians and endosonographers in managing this condition. [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]

Autoimmune pancreatitis (AIP) is a steroid-responsive inflammatory disease of the pancreas. Few studies investigated pancreatic exocrine function (PEF) in patients suffering from AIP and no definitive data are available on the effect of steroids in PEF recovery. Aim of the study is the evaluation of severe pancreatic insufficiency (sPEI) prevalence in AIP at clinical onset and after steroid treatment. 312 Patients with diagnosis of AIP between January 1st, 2010 and December 31st, 2020 were identified in our prospectively maintained register. Patients with a pre-steroid treatment dosage of fecal elastase-1 (FE-1) were included. Changes in PEF were evaluated in patients with available pre- and post-treatment FE (between 3 and 12 months after steroid). One-hundred-twenty-four patients were included, with a median FE-1 of 122 (Q1-Q3: 15–379) μg/g at baseline. Fifty-nine (47.6 %) had sPEI (FE-1<100 μg/g). Univariable analysis identified type 1 AIP, radiological involvement of the head of the pancreas (diffuse involvement of the pancreas or focal involvement of the head), weight loss, age and diabetes as associated with a greater risk of sPEI. However, at multivariable analysis, only the involvement of the head of the pancreas was identified as independent risk factor for sPEI. After steroids, mean FE-1 changed from 64 (15–340) to 202 (40–387) μg/g (P = 0.058) and head involvement was the only predictor of improvement of sPEI. The inflammatory involvement of the head of the pancreas is associated with PEF severity, as well as PEF improvement after treatment with steroids in patients with AIP. [ABSTRACT FROM AUTHOR]

Introduction: IgG4-related disease (IgG4-RD) is a rare disease entity in India. We aimed at studying the clinical profile of IgG4-RD of gastrointestinal tract (GIT) from our centre, while systematically reviewing data from India. Methods: Retrospective review of IgG4-RD of GIT was done using electronic medical records between January 2013 and July 2022. Literature search was done for studies of IgG4-RD of the GIT reported from India from 2000 till January 2023. Case series, case reports of IgG4-RD of GIT and case reports describing GIT with multi-organ involvement were included in the review. Primary outcome measure was response to treatment. Secondary outcome measure was relapse after remission. Results: Thirty-one patients were included with 71% (22/31) having autoimmune pancreatitis. The diagnosis was achieved on surgical specimen in 35% (11/31) patients. Steroid was given to 64% (20/31) patients with remission achieved in 70% (14/20) patients. Four patients exhibitted response to prolonged course of steroids with maintenance azathioprine. Relapse was seen in four (20%) patients who achieved remission. Of 731 articles screened, 48 studies (four case series and 44 case reports) were included in the literature review. Of 95 patients described, steroids were given to 65.2% (62/95), while surgery was done in 33.6% (32/95). Remission was seen in 96.6% (85/88) with relapse occurring in 11.4% (10/88) patients on follow-up. Conclusion: One-third patients of IgG4-RD of GIT are diagnosed after surgery. Response to steroids is good with relapse occurring in up to 12% patients. [ABSTRACT FROM AUTHOR]

Introduction IgG4-related disease (IgG4-RD) is a progressive and potentially life-threatening condition characterized by immune system activation and tissue fibrosis, affecting various organs such as the pancreas, kidneys, and lungs. Initially recognized in 2003, it often presents as mass-like lesions, which can mimic tumors. Despite advancements in understanding its pathology, epidemiological data are limited, and many patients remain undiagnosed due to unfamiliarity with the disease. Aim of the study The aim of this article wast to summarize the latest knowledge on the diagnosis and clinical manifestations of IgG4-related disease. Materiał and methods This review is based on articles from the PubMed and Google Scholar databases, covering the years 2007-2024, using the keywords: IgG4-related disease; autoimmune pancreatitis; retroperitoneal fibrosis; classification criteria. Results The disease's complex pathogenesis involves B and T cell activity, with genetic and environmental factors contributing. While effective treatments, like B cell depletion, exist, the disease's broad clinical manifestations and multi-organ involvement require a multidisciplinary approach for proper diagnosis and management. Conclusion Diagnosing IgG4-RD is challenging due to its varied symptoms, often mimicking other diseases. Key findings include high IgG4 levels and specific histopathological features. Further research is needed to understand its genetic factors, pathogenesis, and epidemiology.

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.

The aim: To present the state-of-the-art of clinical features, differential diagnosis and treatment of IgG4-related sclerosing cholangitis.Key points: IgG4-sclerosing cholangitis is a fibrotic inflammatory disease affecting the intrahepatic and extrahepatic bile ducts. The clinical features of IgG4-sclerosing cholangitis are similar to those of primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. More than one third of patients with IgG4-sclerosing cholangitis undergo surgery. Currently, there are no specific and sensitive methods to diagnose this disease. Increased serum IgG4 levels are observed in many other diseases. A fourfold increase in serum IgG4 levels is a more reliable marker, but this feature is found in only a small percentage of patients. The imaging of bile ducts usually reveals segmental or extended strictures with prestenotic dilatation and wall thickening. Glucocorticosteroids are the first-line therapy for induction and maintenance of disease remission. More than a half of patients develop relapses. Several studies have found an increased risk of malignant tumors. This review describes the clinical, laboratory, and instrumental features of IgG4-sclerosing cholangitis. Comparative evaluation of diseases manifestations versus primary sclerosing cholangitis and cholangiocarcinoma is presented along with options of therapy, prognosis and outcomes of the disease.Conclusion: IgG4-sclerosing cholangitis is a rare and difficult to diagnose disease that requires careful differential diagnosis with primary sclerosing cholangitis, bile duct cancer and pancreatic cancer. Despite its relatively benign course and efficacy of glucocorticosteroid therapy, the disease recurs frequently and has an unknown long-term outcome. Special attention is paid to the risk of malignant neoplasms in this group of patients, emphasizing the need for lifelong follow-up.

Introduction: Autoimmune pancreatitis (AIP) is recognized as a disease with a good prognosis that responds well to steroids, but the complication of pancreatic ductal adenocarcinoma (PDAC) in AIP is a rare condition. We report a case of PDAC encapsulated by tumor-forming type 1 AIP. Case Presentation: The patient, a 65-year-old female, was found to have high CA19-9 levels and a pancreatic mass with a diameter of 30 mm on abdominal ultrasonography. Contrast-enhanced computed tomography revealed a 40-mm mass in the tail of the pancreas that had a 27-mm oligemic mass inside it. From these work-up examinations, this tumor was diagnosed as PDAC, with evidence of colonic invasion. As curative resection for PDAC, a distal pancreatectomy with splenectomy and combined colon resection were performed. Histopathological examination showed invasive PDAC surrounded by IgG4-positive plasma cell infiltration. Based on these findings, a diagnosis was made of PDAC located in the pancreatic tail capsulized by type 1 AIP. The postoperative course was uneventful, and the patient was discharged on postoperative day 15. She underwent postoperative adjuvant chemotherapy with S-1 for 6 months, and no recurrence was noted for 2 years after operation. Conclusion: Currently, there are two hypothetical mechanisms of PDAC induction by AIP: (1) carcinogenic stimulation due to chronic inflammation and (2) paraneoplastic syndrome caused by AIP. Further study of the relationship between AIP and pancreatic cancer is needed, and follow-up should be conducted while keeping in mind the possibility of complications.

Magnetic resonance imaging (MRI) has good diagnostic performance and causes no radiation damage, making it an ideal tool for the autoimmune pancreatitis (AIP) surveillance. However, its time cost is high. This study aimed to evaluate (1) whether a simplified protocol (SP) of MRI for AIP surveillance provides information equivalent to the comprehensive protocol (CP) and (2) the time cost reductions associated with using an SP. This retrospective single-institutional study included 40 patients with AIP with at least two contrast-enhanced MRI/magnetic resonance cholangiopancreatography studies. Two radiologists evaluated two imaging sets (CP/SP) per patient, independently. Intra- and inter-observer agreement in the evaluation of the pancreas and extrapancreatic organs involvement using the SP/CP in addition to the time cost differences between the SP and CP were assessed. Intra- and inter-rater reliability were assessed using Cohen's kappa test, intraclass correlations, or the weighted kappa test. The differences in time costs between the CP and SP were compared using the Mann-Whitney U test or Wilcoxon signed-rank test. The SP had strong intra- and inter-observer agreement with the CP in evaluating MRI parameters (κ ＞ 0.60, moderate to excellent) and disease activity status (κ ＞ 0.80, all excellent). The overall image acquisition time cost for the SP was 49.2% of the CP. For the two radiologists, the image interpretation time cost of the SP was reduced by approximately 35% and 27% compared to the CP. For AIP surveillance, SP MRI provides information consistent with the CP and is less time-consuming. [ABSTRACT FROM AUTHOR]

Autoimmune Pancreatitis (AIP) is a rare chronic inflammatory disease affecting the pancreas. Chronic pancreatic inflammation represents a risk factor for pre-neoplastic conditions such as Intraductal Papillary Mucinous Neoplasia (IPMN). Due to the rarity of AIP, the incidence, and clinical features of IPMN occurring in AIP patients remains unknown. In the present study we aimed to explore the relationship between AIP and IPMN and to characterize the clinical features and outcomes of IPMN occurring in the context of AIP. We retrospectively (2008–2020) analyzed the clinical and radiological records of a large single center cohort of patients with AIP and investigated the prevalence of IPMN. We then compared the clinical, laboratory and radiological characteristics of patients with IPMN and AIP with a cohort of patients with isolated IPMN. Five hundred and nineteen patients were included in this retrospective study. Sixteen patients had concomitant IPMN and AIP(3%); 61 patients had isolated AIP (12%); 442 patients had isolated IPMN (85%). The prevalence of IPMN in patients with AIP was higher than that observed in the general population (21%vs8-10%). Worrisome Features and High-Risk Stigmata were more frequently observed in IPMN occurring together with AIP compared to isolated IPMN(p < 0.05). Based on radiological features IPMN in the context of AIP was more frequently of main-duct type compared to isolated IPMN(p < 0.05). Our data suggest that AIP represents a chronic inflammatory condition that might favor IPMN development with high-risk features. Prolonged surveillance of these patients and longitudinal studies are required to further test the association with AIP and malignant and pre-malignant conditions. [ABSTRACT FROM AUTHOR]

The association between autoimmune pancreatitis (AIP) and pancreatic cancer (PC) remains controversial. This study aimed to clarify the long-term prognosis and risk of malignancies in AIP patients in Japan. We conducted a multicenter retrospective cohort study on 1364 patients with type 1 AIP from 20 institutions in Japan. We calculated the standardized incidence ratio (SIR) for malignancies compared to that in the general population. We analyzed factors associated with overall survival, pancreatic exocrine insufficiency, diabetes mellitus, and osteoporosis. The SIR for all malignancies was increased (1.21 [95 % confidence interval: 1.05–1.41]) in patients with AIP. Among all malignancies, the SIR was highest for PC (3.22 [1.99–5.13]) and increased within 2 years and after 5 years of AIP diagnosis. Steroid use for ≥6 months and ≥50 months increased the risk of subsequent development of diabetes mellitus and osteoporosis, respectively. Age ≥65 years at AIP diagnosis (hazard ratio [HR] = 3.73) and the development of malignancies (HR = 2.63), including PC (HR = 7.81), were associated with a poor prognosis, whereas maintenance steroid therapy was associated with a better prognosis (HR = 0.35) in the multivariate analysis. Maintenance steroid therapy was associated with a better prognosis even after propensity score matching for age and sex. Patients with AIP are at increased risk of developing malignancy, especially PC. PC is a critical prognostic factor for patients with AIP. Although maintenance steroid therapy negatively impacts diabetes mellitus and osteoporosis, it is associated with decreased cancer risk and improved overall survival. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis is a sub-type of chronic pancreatitis. It has 2 further types; type I and II. Type I is associated with IgG4 disease. We describe a case of chronic pancreatitis that ultimately turned out to be IgG4 disease. A 45-year-old female with no co-morbidities presented on 18th November 2022 with epigastric pain, left hypochondrium pain, vomiting, and fever for 5 days. She had previously been admitted twice to the hospital with similar complaints and was diagnosed with acute pancreatitis. Examination revealed tenderness in the epigastrium and left hypochondrium; Laboratory investigations revealed serum Lipase 405 U/L, serum Amylase; 178 U/L, Lipid profile. cholesterol; 242 mg/dL triglycerides; 218 mg/dL HDL; 42 mg/dL LDL; 169 mg/dL. HbA1c; 9.53%. and IgG4 level; 1657mg/liter. CT abdomen with pancreatic protocol showed a cystic lesion in the body and tail of the pancreas suggestive of an infected pseudocyst or localized acute pancreatitis. The patient was diagnosed with type 1 autoimmune pancreatitis and a multidisciplinary team (MDT) was taken on board for further management. The patient was not deemed appropriate for percutaneous or endoscopic drainage of the pseudocyst. She therefore underwent distal pancreatectomy and splenectomy. The biopsy showed features favoring mucinous cystic neoplasm of the pancreas with no evidence of malignancy. On immunohistochemical stains, the reactivity pattern was positive for ER, inhibin, and CD 10 which highlighted the ovarian stroma and cytokeratin. The patient was discharged on 25th November 2022 on supportive treatment. [ABSTRACT FROM AUTHOR]

The diagnostic pathway of pancreatic lesions has changed since endoscopic ultrasound-guided biopsies were introduced. The tissue diagnosis of pancreatic ductal adenocarcinoma (PDAC) used to be made by cytology or surgical specimens, while pancreatic biopsies currently contribute to a pretreatment diagnosis in >80 % of cases. Pathologists need to assess subtle changes in biopsy specimens, in order to avoid over- and under-diagnosis of PDAC. The lack of stromal reaction does not exclude the possibility of PDAC, while abnormal distribution of the ducts and a mild but sufficient degree of nuclear atypia are diagnostic clues for well differentiated PDAC. It is also important to be aware of distinct types of pancreatitis that potentially presents with a tumour. IgG4-related autoimmune pancreatitis (type 1 AIP) is widely recognised as a mimic of PDAC. Other forms of mass-forming pancreatitis include type 2 AIP (IgG4-unrelated), follicular pancreatitis, groove pancreatitis and acute interstitial pancreatitis. Most of those conditions lack serological diagnostic markers; therefore, the histology plays a central role in the diagnosis. [ABSTRACT FROM AUTHOR]

Background: The risk and prognosis of pancreatobiliary cancer and in patients with autoimmune pancreatitis (AIP) and IgG4‐related sclerosing cholangitis (IgG4‐SC) remain unclear. Therefore, we retrospectively investigated the risk of pancreatobiliary cancer and prognosis in patients with AIP and IgG4‐SC. Methods: Patients with AIP and IgG4‐SC at seven centers between 1998 and 2022 were investigated. The following data were evaluated: (1) the number of cancers diagnosed and standardized incidence ratio (SIR) for pancreatobiliary and other cancers during the observational period and (2) prognosis after diagnosis of AIP and IgG4‐SC using standardized mortality ratio (SMR). Results: This study included 201 patients with AIP and IgG4‐SC. The mean follow‐up period was 5.7 years. Seven cases of pancreatic cancer were diagnosed, and the SIR was 8.11 (95% confidence interval [CI]: 7.29–9.13). Three cases of bile duct cancer were diagnosed, and the SIR was 6.89 (95% CI: 6.20–7.75). The SMR after the diagnosis of AIP and IgG4‐SC in cases that developed pancreatobiliary cancer were 4.03 (95% CI: 2.83–6.99). Conclusions: Patients with autoimmune pancreatitis and IgG4‐SC were associated with a high risk of pancreatic and bile duct cancer. Patients with AIP and IgG4‐SC have a worse prognosis when they develop pancreatobiliary cancer. [ABSTRACT FROM AUTHOR]

The impact of extended steroid administration on patients with autoimmune pancreatitis after a 3-year maintenance period remains poorly understood. This study analyzed the advantage and disadvantage of continuing steroid therapy beyond 3 years. In this retrospective multicenter study across 17 institutions, patients who successfully completed 3 years of maintenance therapy without experiencing relapse were categorized into two groups: the maintenance therapy discontinuation group, who discontinued steroid therapy after the initial 3-year period, and maintenance therapy continuation group, who continued steroid therapy beyond 3 years. The cumulative relapse rate after 3 years of maintenance therapy was the primary outcome. Relapse predictors were compared using the Gray test for cumulative relapse incidence by specific factor. Of 211 patients, 105 experienced no relapse during the 3-year maintenance therapy and were divided into two groups: 69 in the maintenance therapy discontinuation group and 36 in the maintenance therapy continuation group. The relapse rate was lower in the maintenance therapy continuation group than in the maintenance therapy discontinuation group (P = 0.035). Predictors of relapse after 3 years included cessation of maintenance therapy (hazard ratio [HR] = 3.76; 95 % confidence interval [CI] = 1.07–13.3, P = 0.040) and renal involvement (HR = 2.88; 95 % CI = 1.04–7.99, P = 0.042). The maintenance therapy continuation group showed a significantly higher prevalence of macrovascular complications, compared with the maintenance therapy discontinuation group (P = 0.005). Cessation of steroid maintenance therapy and renal involvement were predictors of relapse after 3 years of maintenance therapy. However, the long-term use of steroids may increase the risk of macrovascular complications. [ABSTRACT FROM AUTHOR]

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]

Autoimmune pancreatitis type 2 is a relatively novel entity with some still controversial issues. The current diagnostic algorithm relies on imaging studies and histology. Therapy includes corticosteroids with consequently low risk of relapse in the following year. However, the pathogenesis remains unclear, and data are insufficient for long-term prognosis. We have treated a 17-year-old boy whose autoimmune pancreatitis type 2 was revealed during surgery for a pre-existing biliary tract anomaly with concurrent protozoal infection. We discuss the co-occurrence of these conditions in terms of eventual pathogenesis correlation and combined effect on long-term prognosis.

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Introduction: Autoimmune pancreatitis is a type of chronic autoimmune inflammation that can be divided into two types. Type 1 is a clinical manifestation of IgG4-mediated disease, while type 2 may coexist with inflammatory bowel diseases. Objective: The aim of the study is to review the literature on the role of the intestinal microbiota in the pathogenesis of autoimmune pancreatitis. Methods: A literature review was conducted on databases such as PubMed and Google Scholar using the terms: "autoimmune pancreatits", "intestinal dysbiosis", "intestinal microbiome", and "pathogenesis". Conclusions: The intestinal microbiota has an impact on the development of autoimmune pancreatitis, but it is not the main pathogenic factor, but rather a risk factor. [ABSTRACT FROM AUTHOR]

Introduction: Autoimmune pancreatitis (AIP) is recognized as a disease with a good prognosis that responds well to steroids, but the complication of pancreatic ductal adenocarcinoma (PDAC) in AIP is a rare condition. We report a case of PDAC encapsulated by tumor-forming type 1 AIP. Case Presentation: The patient, a 65-year-old female, was found to have high CA19-9 levels and a pancreatic mass with a diameter of 30 mm on abdominal ultrasonography. Contrast-enhanced computed tomography revealed a 40-mm mass in the tail of the pancreas that had a 27-mm oligemic mass inside it. From these work-up examinations, this tumor was diagnosed as PDAC, with evidence of colonic invasion. As curative resection for PDAC, a distal pancreatectomy with splenectomy and combined colon resection were performed. Histopathological examination showed invasive PDAC surrounded by IgG4-positive plasma cell infiltration. Based on these findings, a diagnosis was made of PDAC located in the pancreatic tail capsulized by type 1 AIP. The postoperative course was uneventful, and the patient was discharged on postoperative day 15. She underwent postoperative adjuvant chemotherapy with S-1 for 6 months, and no recurrence was noted for 2 years after operation. Conclusion: Currently, there are two hypothetical mechanisms of PDAC induction by AIP: (1) carcinogenic stimulation due to chronic inflammation and (2) paraneoplastic syndrome caused by AIP. Further study of the relationship between AIP and pancreatic cancer is needed, and follow-up should be conducted while keeping in mind the possibility of complications. [ABSTRACT FROM AUTHOR]

Ulcerative colitis (UC) is a chronic idiopathic inflammatory disease mainly affecting the rectum and colon and causing diarrhoea and mucopurulent stools. UC can present with extraintestinal manifestations in various organs and systems and can be associated with various comorbidities. Autoimmune pancreatitis (AIP) is a specific type of pancreatitis associated with autoimmune abnormalities and is divided into two clinical types: type 1 (lymphoplasmacytic sclerosing pancreatitis) and type 2 (idiopathic ductocentric pancreatitis). The current study shows an association between type 2 AIP and UC, which may be related to genetic susceptibility, inflammatory factors, and immune response. The most common manifestation of AIP in patients with type 2 AIP-UC is abdominal pain with elevated pancreatic enzymes, whereas the presentation of UC in type 2 AIP-UC is more severe, with an increased risk of UC-related surgery. This review focuses on diagnosis, prevalence, pathogenesis, impact, and treatment to better understand type 2 AIP-UC, explore the molecular mechanisms of this condition, and encourage further research into the management of type 2 AIP-UC. [ABSTRACT FROM AUTHOR]

IgG4-related cholangitis (IRC) is the major hepatobiliary manifestation of IgG4-related disease (IgG4-RD), a systemic fibroinflammatory disorder. The pathogenesis of IgG4-RD and IRC is currently viewed as multifactorial, as there is evidence of a genetic predisposition while environmental factors, such as blue-collar work, are major risk factors. Various autoantigens have been described in IgG4-RD, including annexin A11 and laminin 511-E8, proteins which may exert a partially protective function in cholangiocytes by enhancing secretion and barrier function, respectively. For the other recently described autoantigens, galectin-3 and prohibitin 1, a distinct role in cholangiocytes appears less apparent. In relation to these autoantigens, oligoclonal expansions of IgG4+ plasmablasts are present in patients with IRC and disappear upon successful treatment. More recently, specific T-cell subtypes including regulatory T cells, follicular T helper 2 cells, peripheral T helper cells and cytotoxic CD8+ and CD4+ SLAMF7+ T cells have been implicated in the pathogenesis of IgG4-RD. The clinical presentation of IRC often mimics other biliary diseases such as primary sclerosing cholangitis or cholangiocarcinoma, which may lead to inappropriate medical and potentially invalidating surgical interventions. As specific biomarkers are lacking, diagnosis is made according to the HISORt criteria comprising histopathology, imaging, serology, other organ manifestations and response to therapy. Treatment of IRC aims to prevent or alleviate organ damage and to improve symptoms and consists of (i) remission induction, (ii) remission maintenance and (iii) long-term management. Glucocorticosteroids are highly effective for remission induction, after which immunomodulators can be introduced for maintenance of remission as glucocorticosteroid-sparing alternatives. Increased insight into the pathogenesis of IRC will lead to improved diagnosis and novel therapeutic strategies in the future. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Total signs of Raghib syndrome in AIP case accompanied by an IgG4‐related disease were described. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis scarcely found in children. Raghib syndrome is a rare congenital heart defect known as persistent left superior vena cava (LSVC) draining into the left atrium. Here, we describe Raghib syndrome in AIP case accompanied by an IgG4‐related disease (AIP/IgG4RD). A 13‐year‐old boy presented with a 3‐month history of fever and abdominal pain. The laboratory findings showed SGOT and SGPT, ALP was increased, while amylase and γ‐GT were normal. Immunoglobulins were normal, except for IgG. Endosonography, spiral CT of the abdomen, and cholangiopancreatography showed an enlargement of the pancreas. Contrast echocardiography discovered opacification of the coronary sinus and left atrium. Transesophageal echocardiography for LSVC revealed a dilatation in the coronary sinus, indicating persistent LSVC. Following the injection of agitated saline into the left antecubital vein, bubbles entered both left and right atria in LSVC. It is reasonable to exclude some of these rare disorders as Raghib syndrome, in cases that will be started on medications like corticosteroids, which increases the susceptibility to thromboembolic events. [ABSTRACT FROM AUTHOR]