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ABSTRACT Aims/Introduction To investigate factors influencing glycemic control in diabetes mellitus complicated by autoimmune pancreatitis. Materials and Methods This retrospective cohort study investigated 33 patients with diabetes mellitus complicated by autoimmune pancreatitis who had received steroid therapy at Toranomon Hospital between January 1, 2011, and December 31, 2020. The course of glycemic control at 12 months after starting steroids was classified into three groups: Improved, Unchanged, or Worsened. Factors affecting these groups were investigated. Furthermore, we created two scores: (1) time of diabetes mellitus onset and baseline body mass index; (2) time of diabetes mellitus onset and baseline C‐peptide index. Diabetes mellitus occurring at the same time as autoimmune pancreatitis, body mass index ≥22 kg/m2, and C‐peptide index ≥1.1 were each worth 1 point. Scores were summed and totals of 0–2 were compared between groups. Results Ten patients were in the Improved group, 10 were in the Unchanged group, and 13 were in the Worsened group. The baseline body mass index and baseline C‐peptide index were lower in the Worsened group than in the Improved group (P

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.

Autoimmune pancreatitis responds well to corticosteroids in most instances. Additional immunosuppression or low-dose maintenance steroids may be necessary upon relapse. There is limited data on alternative strategies when these regiments fail or cause adverse reactions. We report a case of a middle-aged woman with autoimmune pancreatitis in whom tapering of prednisolone below the dose of 25mg per day resulted in relapse of symptoms and long-term steroid use led to development of steroid induced hyperglycaemia. Induction and maintenance of steroid-free remission was ultimately successful under vedolizumab therapy. Remission has been stable for over one year with reduced need for antidiabetic intervention. This is the first reported case of treatment of refractory autoimmune pancreatitis with vedolizumab. It highlights the overlap of immunological mechanisms within inflammatory diseases of the digestive tract and how knowledge of biological data can inform treatment decisions for individual cases. The demonstrated efficacy of vedolizumab and low risk of severe side effects warrant further investigation into its use in autoimmune pancreatitis.

IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multiorgan involvement. Autoimmune pancreatitis (AIP) is classified into two types: type 1 AIP as a pancreatic manifestation of IgG4-RD and type 2 AIP with granulocytic epithelial lesion and occasional association with ulcerative colitis. Although the pathogenic mechanism still remains unclear, possible multipathogenic factors such as genetic factors, disease-specific or related antigens, and abnormal innate or adaptive immunity may be involved in the development of IgG4-RD. Many immunocytes including M2 macrophages, plasmablasts, B cells, and T-cells (Th2-CD4+T, follicular helper T-cells, and CD4+SLAMF++cytotoxic T-cells) play important roles in the pathogenesis. Conventional induction and maintenance therapies with glucocorticoid or rituximab are recommended in all symptomatic patients with active IgG4-RD. In those at risk for irreversible damage in any organs, this should be done urgently, regardless of symptoms. As no randomized clinical trials other than glucocorticoid maintenance therapy for type 1 AIP have been performed, the comprehensive management for IgG4-RD has not been established yet. Targeted treatment approaches against the plasmablast to B cell lineage and the CD4+ SLAMF7+ cytotoxic T-cell seem to be promising for the future-directed treatment. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis is a rare form of chronic pancreatitis of presumed autoimmune etiology. Due to significant overlap in clinical and imaging characteristics, misdiagnosis as a pancreatic malignancy is common. As a result, a significant number of patients undergo a major pancreatic resection, associated with considerable morbidity, for a disease process that generally responds well to corticosteroid therapy. In the past ten years, important advances have been made in understanding the disease. Several diagnostic criteria have been developed to aid in diagnosis. Despite this, pancreatic resection may still be required in a subset of patients to reliably exclude pancreatic malignancy and establish a definite diagnosis of autoimmune pancreatitis. This article aimed to define the role of surgery in autoimmune pancreatitis, if any. For this purpose, published case series of patients with a diagnosis of autoimmune pancreatitis, based on the histopathological examination of surgical specimens, were reviewed and patients' clinical, radiological and serological details were assessed. At the end, histopathologic examinations of patients who underwent pancreatic resection at our department in the last 10 years were retrospectively reviewed in order to identify patients with autoimmune pancreatitis and assess their clinical characteristics. [ABSTRACT FROM AUTHOR]

A 70-year-old man presented with unprovoked weight loss and intermittent left upper quadrant tenderness for one-and-a-half month. Computed tomography revealed irregular cystic lesions in the pancreatic tail. Endoscopic ultrasonography (EUS) revealed pancreatic tail enlargement and giant, thick-walled cysts surrounding the pancreatic tail with no connection with the pancreatic duct. Endoscopic ultrasonography-guided fine-needle aspiration was performed on the enlarged pancreatic parenchyma and thickened cyst wall. Both biopsies showed hyper eosinophilia and few plasma cells. Endoscopic retrograde pancreatography revealed an irregular narrowing of the main pancreatic duct. Pancreatic juice cytology revealed substantial eosinophilia. Blood sampling showed an elevated eosinophil count and immunoglobulin G4 (IgG4) and immunoglobulin E (IgE) levels at the initial examination. We considered the patient to have eosinophilic pancreatitis (EP) with autoimmune pancreatitis, or alternately, EP with IgG4-related retroperitoneal fibrosis (RPF). Upon prednisolone administration, the abdominal pain improved, the peripheral blood eosinophil count decreased to zero, IgG4 and IgE levels decreased, pancreatic enlargement improved, and the cystic lesions disappeared. The condition did not recur within the following 3 years. Both EP and EP with IgG4-related RPF are rare etiologies of pancreatitis, and this case is very instructive. [ABSTRACT FROM AUTHOR]

A 65-year-old woman presented with epigastric pain persisting for more than 3 months. She was diagnosed with autoimmune pancreatitis (AIP), based on high serum IgG4 levels (981 mg/dL) and diffuse pancreatic enlargement with a capsule-like rim on computed tomography (CT). Additionally, the main pancreatic duct was indistinct on magnetic resonance cholangiopancreatography. CT, esophagogastroduodenoscopy, and upper gastrointestinal radiography revealed stenosis with gastric outlet obstruction (GOO) in the second part of the duodenum. Prednisolone administration was initiated as treatment; on day 3 of treatment, the patient's symptoms improved. After 2 weeks, CT and endoscopic ultrasonography of the duodenal bulbs revealed improvement of the enlarged pancreas. The second part of the duodenum ran into the pancreatic head, and no malignant lesions were observed. Based on the above findings, we suspect that she developed AIP in the annular pancreas (AnnP), where duodenal stenosis worsened with diffuse pancreatic enlargement, resulting in GOO. She is currently under careful observation with tapering of prednisolone—without surgical treatment for AnnP. The pathogenesis of GOO caused by AIP without malignancy is rare. One case of GOO caused by AIP, wherein AIP developed in the AnnP (similar to the present case), has been reported, highlighting the novelty of our report. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) has attracted much attention in the last two decades, and due to the diagnostic value of immunoglobulin G4 (IgG4), the number of cases diagnosed in clinical practice has markedly increased. However, in contrast to prototypic IgG4-related type 1 AIP, a minor subtype of AIP, referred to as type 2 AIP, is less widely known and has thus not yet been characterized in detail. Type 2 AIP is unrelated to IgG4 and is a completely distinct entity from type 1 AIP. One confusing factor is that the two types of AIP share patterns of clinical presentation (e.g., acute pancreatitis and painless jaundice) and imaging abnormalities (e.g., diffuse or segmental enlargement). Since there are currently no established serum markers, the diagnosis of type 2 AIP is highly challenging and requires the tissue confirmation of neutrophilic injury to the pancreatic ducts, a finding designated as a granulocytic epithelial lesion. Approximately one-third of cases are associated with inflammatory bowel disease, particularly ulcerative colitis; however, the pathological relationship between these two conditions has not yet been clarified. Unanswered questions relate to its pathophysiology, the potential development of a similar granulocytic injury in other organs, and the characteristics of pediatric cases. This review summarizes consensus and controversies surrounding type 2 AIP, with the aim of increasing awareness and highlighting the unmet needs of this underrecognized condition.

Background/Aims Mass-forming autoimmune pancreatitis may be difficult to differentiate from pancreatic cancer. Endoscopic ultrasound-guided tissue acquisition (EUS-TA) and a steroid trial can be helpful in diagnosis. Here, we report a case of mass-forming autoimmune pancreatitis that was challenging to distinguish from pancreatic head cancer. Case Presentation An 81-year-old male presented with obstructive jaundice and a 2 cm pancreatic head mass. His medical history included hypertension. Before visiting our institution, he had undergone abdominal computed tomography (CT) with contrast enhancement, endoscopic retrograde cholangiopancreatography (ERCP) with biliary stent placement, and EUS-guided fine needle biopsy (EUS-FNB). Cytology and endobiliary biopsy were negative for malignancy. CT at our institution showed a slight decrease in the pancreatic head mass, raising suspicion of autoimmune pancreatitis. Serum IgG4 was elevated at 1247 mg/L (normal range: 50-1200 mg/L). Antinuclear antibody (ANA) titer was 1:40, and carbohydrate antigen 19-9 (CA 19-9) was 5 U/mL (normal range: <37 U/mL). ERCP revealed focal obstruction in the distal bile duct. After cytology and endobiliary biopsy, a new plastic biliary stent was placed, and EUS-FNB was repeated. Pathology showed chronic inflammation, but IgG4 staining was negative. Given the inconclusive results, a 2-week steroid trial with 50 mg of oral prednisolone was initiated. Prednisolone was tapered by 5 mg every 2 weeks. Follow-up CT revealed a decreased size of the pancreatic mass to 1.3 cm. Follow-up ERCP showed improved bile duct obstruction, eliminating the need for the stent. A subsequent CT after 2 months confirmed further reduction in the pancreatic mass. The patient remains asymptomatic and is on a maintenance dose of 5 mg prednisolone. Conclusion This case underscores the challenge of differentiating mass-forming autoimmune pancreatitis from pancreatic cancer. [ABSTRACT FROM AUTHOR]

Background/Aims Patients with ulcerative colitis (UC) are at increased risk of acute pancreatitis. Autoimmune pancreatitis (AIP) is considered an autoimmune disease and is divided into type 1 and type 2. Type 2 AIP primarily affects the pancreas and is not associated with IgG4. In addition, type 2 AIP has no specific biomarkers and usually requires pancreatic histology. Here we present a UC patient who was diagnosed with type 2 AIP after steroid treatment. Case Presentation A 34-year-old woman presented with fever and abdominal pain. She was diagnosed with UC (left-sided colitis) 3 years ago and treated with combined oral and rectal 5-aminosalicylic acid. She underwent laparoscopic cholecystectomy for acute cholecystitis 1 year ago. For 1 month she had frequent mucoid stools, fever and periumbilical abdominal pain. Acute pancreatitis was diagnosed on CT scan. Serum lipase was elevated to 897 U/L. MRCP showed no evidence of stones or malignancy. The IgG4 subclass level was normal (12.6 mg/dL) and autoimmune markers such as anti-mitochondrial Ab, anti-smooth muscle Ab and ANCA were all negative. Endoscopic examination revealed active inflammation in the descending and sigmoid colon. She refused pancreatic biopsy, so with the probable diagnosis of type 2 AIP in active UC, oral steroid treatment was started and her symptoms improved dramatically. After 2 weeks of steroid treatment, the lipase level was reduced to 119 U/L. Conclusion The association between type 2 AIP and UC is well established, but whether type 2 AIP is an extra-intestinal manifestation of UC is unclear. Patients with active UC presenting with acute pancreatitis could be diagnosed with probable type 2 AIP without histology if the pancreatitis improves rapidly after steroid treatment. An understanding of the clinical relevance and potential pathogenesis of type 2 AIP UC may help to guide the appropriate treatment. [ABSTRACT FROM AUTHOR]

[Display omitted] Enhanced IgG4 antibody (Ab) response is a prominent feature of type 1 autoimmune pancreatitis (AIP). Innate immune responses associated with IgG4 Ab production are poorly defined. We have previously reported that peripheral blood mononuclear cells (PBMCs) isolated from patients with type 1 AIP produce large amounts of IgG4 Abs upon stimulation with bacterial cell wall components. In addition, we showed that activation of plasmacytoid dendritic cells producing interferon (IFN)-α, interleukin (IL)-33, and B cell-activating factor (BAFF) upon sensing intestinal bacteria mediates the development of experimental AIP. In this study, we attempted to clarify the role of innate immunity against fungi in inducing enhanced IgG4 Ab responses in type 1 AIP. PBMCs isolated from healthy controls and patients with type 1 AIP were stimulated with a broad range of bacterial and fungal cell wall components. The concentrations of IgG1, IgG4, and cytokines were measured using enzyme-linked immunosorbent assays. Cell wall components derived from bacteria and fungi induced IgG1 and IgG4 Ab production in patients with type 1 AIP. Various types of microbe-associated molecular pattern motifs enhanced IgG4 Ab production in patients with type 1 AIP compared with the limited motifs in healthy controls. The enhanced IgG1 and IgG4 Ab production that followed in response to bacterial and fungal cell wall components was parallel to that of IFN-α, IFN-γ, IL-10, IL-33, and BAFF. In conclusion, cell wall components derived from fungi as well as bacteria promote IgG4 Ab responses in patients with type 1 AIP. [ABSTRACT FROM AUTHOR]

IgG4-related sclerosing cholangitis (IgG4-SC) can be classified into four types based on cholangiographic findings and regions of biliary stricture. This cholangiographic classification is useful to differentiate IgG4-SC from mimickers including cholangiocarcinoma, primary sclerosing cholangitis, and pancreatic cancer. Autoimmune pancreatitis (AIP) is a valuable clue for the diagnosis of IgG4-SC because the two are frequently found in association with each other. Two sets of diagnostic criteria for IgG4-SC have been proposed. In Japan, the clinical diagnostic criteria 2020 were recently developed. These clinical diagnostic criteria include narrowing of the intrahepatic and/or extrahepatic bile duct, thickening of the bile duct wall, serological findings, pathological findings, other organ involvement, and effectiveness of steroid therapy. When these criteria are applied, IgG4-SC is initially classified as associated or not associated with AIP, and cholangiographic classification is used for differential diagnosis. In most instances, IgG4-SC can be diagnosed on the basis of clinical diagnostic criteria. However, it is challenging to diagnose isolated IgG4-SC or IgG4-SC not associated with AIP. Here, we review the classification and diagnostic criteria for IgG4-SC, specifically focusing on the clinical diagnostic criteria 2020 and a large IgG4-SC case series from a nationwide survey in Japan.

Autoimmune pancreatitis (AIP) is a rare, often‐missed disease that involves inflammation of the pancreas and strictures of the pancreatic duct. Its prevalence and incidence in the United States remain scarce. The disease has a varied presentation and often mimics pancreatic malignancy, which can make the diagnosis challenging. Most patients have an excellent response to corticosteroid therapy. Immunomodulators may be used in some cases. Rituximab is an effective, emerging treatment in steroid‐refractory cases. This study aims to review the two distinct types of AIP and provide a detailed analysis of the diagnostic approach and treatment modalities.

Background & Aims: Type 1 autoimmune pancreatitis (AIP) is an IgG4-related disease whose diagnosis is challenging. The aim of this study was to investigate the diagnostic value of circulating total and IgG4+ plasmablasts in differentiating this condition from the other main pancreatic diseases. Methods: Patients with type 1 AIP (n = 19) were prospectively enrolled in a tertiary center together with patients suffering from type 2 or not otherwise specified (NOS) AIP (n = 10), pancreatic adenocarcinoma (n = 17), chronic pancreatitis (n = 20), and intraductal papillary mucinous neoplasia or chronic asymptomatic pancreatic hyperenzymemia (n = 21) as control groups. Flow cytometry was used to measure the total plasmablast and IgG4+ plasmablast number by gating peripheral blood CD45+CD19+CD38hiCD20-CD24-CD27+ and CD45+CD19+CD38hiCD20-CD24-CD27+IgG4+ cells, respectively. In patients with AIP, these cell populations were also evaluated after 1 month of therapy, after 2–4 months from the end of treatment, and after 1 year from the enrollment. The study was approved by the local ethics committee (protocol number: 59133, 30/11/2017). Results: Total plasmablast quantification was capable of discriminating type 1 AIP from all the other pancreatic disorders with a sensitivity of 47% and a specificity of 81%, according to a cutoff of 4500 cells/mL (AUC = 0.738), whereas IgG4+ plasmablast count distinguished type 1 AIP from all the other pancreatic disorders with a sensitivity of 80% and a specificity of 97% when applying a cutoff of 210 IgG4+ cells/mL (AUC = 0.879). The basal IgG4+ plasmablast number was significantly higher (P = .0001) in type 1 AIP than in type 2/NOS AIP, decreased after steroid therapy, and increased at disease relapse. Conclusion: IgG4+ plasmablast count represents a potentially useful biomarker to differentiate type 1 from type 2/NOS AIP and from other pancreatic diseases.

COVID-19 exhibits diverse and systemic clinical symptoms, much like systemic autoimmune diseases, and there are notable similarities in the immune responses seen in both conditions. There are rare reports of ulcerative colitis and autoimmune hepatitis triggered by COVID-19 infection. Reported herein is a case of a previously healthy patient who was diagnosed with chronic colitis resembling ulcerative colitis, autoimmune pancreatitis, and suspected immune-mediated hepatitis (AIH-like hepatitis) 2 months after a COVID-19 infection. A 33-year-old COVID-19-vaccinated male, presented with abdominal pain, nausea, and vomiting for 2 days. He also had bloody diarrhea that persisted for 2 months after recovering from a COVID-19 infection. A diagnosis of acute pancreatitis was confirmed by markedly elevated serum amylase and lipase and a CT scan of the abdomen. Colonoscopy and histopathology findings also confirmed a diagnosis of chronic colitis resembling ulcerative colitis (Mayo Endoscopy Subscore 3). Marked improvement in bloody diarrhea was observed within 72 h of treatment with IV prednisolone. MRI of the abdomen performed due to an unresolved clinical picture of pancreatitis revealed a bulky pancreas showing delayed diffuse homogenous enhancement, findings possibly consistent with autoimmune pancreatitis. Investigation for elevated liver transaminases showed high titers of antinuclear antibodies and anti-smooth muscle (anti-actin) antibodies while viral hepatitis markers were negative. The patient had already been started on steroid therapy before the lab results were available, with rapid normalization of liver enzymes following treatment. A liver biopsy was not performed. The patient is currently on mesalazine 4 gr/day, and azathioprine 100 mg/day – oral steroids had been tapered and discontinued. Seven months after the initial diagnosis, the patient remains symptom-free. A high level of suspicion for autoimmune disorders is required when assessing patients with a history of COVID-19 infection, although diagnostic pathways remain the same, with generally good response and remission rates to conventional treatment.

Background/ObjectivesAutoimmune pancreatitis (AIP) is a distinct form of pancreatic inflammatory disease that responds well to glucocorticoid therapy. Knowledge on AIP has rapidly evolved over the past two decades. Based on bibliometric analysis, this study aimed to assess the research status of AIP over the past two decades and determine the research focus and emerging topics.MethodsAIP-related publications published between January 1, 2002, and June 6, 2022, were retrieved from the Web of Science Core Collection. Bibliometric data were analyzed using HisCite, VOSviewer, CiteSpace, and bibliometrix package. Annual output, leading countries/regions, active institutions and authors, core journals and references, and keywords of AIP were evaluated.ResultsOverall, 1,772 publications were retrieved from 501 journals by 6,767 authors from 63 countries/regions. Japan published articles on AIP the most (n=728, 41.1%), followed by the United States (n=336, 19%), Germany (n=147, 8.3%), China (n=127, 7%), and Italy (n=107, 6%). The top three most prolific authors were Terumi Kamisawa from Tokyo Metropolitan Komagome Hospital (n=117), Kazuichi Okazaki from Kansai Medical University (n=103), and Shigeyuki Kawa from Matsumoto Dental University (n=94). Pancreas was the most productive journal regarding AIP research (n=95), followed by the Journal of Gastroenterology (n=67), Internal Medicine (n=66), Pancreatology (n=63), and World Journal of Gastroenterology (n=62). “Diagnosis” was the most mentioned keyword. “Risk,” “malignancy,” “outcome,” “22-gauge needle,” and “fine-needle aspiration” were recognized as emerging topics.ConclusionJapan was the leading country in AIP research. Research papers were mainly published in specialized journals. Diagnosis was the research focus. Long-term outcomes and pancreatic tissue acquisition were recognized as research frontiers for AIP.

ObjectiveThis study aimed to explore the clinical characteristics and differential diagnosis of patients with autoimmune pancreatitis (AIP) and pancreatic cancer to prevent misdiagnosis and mistreatment.MethodsThe clinical data of patients with AIP with suspected pancreatic or bile duct malignancy and pancreatic cancer were retrospectively analyzed. The risk factors and the diagnostic value of IgG4 and Tbil levels before treatment for AIP was investigated. Moreover, the imaging features and response to hormone therapy were analyzed.ResultsAIP was commonly observed in men. Compared to patients with pancreatic cancer, the incidence of poor appetite and weight loss and carbohydrate antigen 19-9 (CA19-9) level was lower in patients with AIP, while the immunoglobulin G4 (IgG4) level was higher (p

OBJECTIVES:Autoimmune pancreatitis (AIP) represents a complex immune-mediated pancreas disorder. Pediatric AIP (P-AIP) is rare. We have recently summarized the characteristic features of P-AIP. We now aim to develop recommendation statements to standardize the diagnostic and therapeutic approach to P-AIP and facilitate future research in the field. METHODS:A panel of pediatric gastroenterologists participating in the International Study Group of Pediatric Pancreatitis: In search for a cuRE was formed to discuss and then vote on 15 recommendation statements. A consensus of at least 80% was obtained following 3 voting rounds and revision of the statements. RESULTS:We have now generated 15 statements to help standardize the approach to diagnosis and management of P-AIP. CONCLUSIONS:The first P-AIP recommendation statements developed by the International Study Group of Pediatric Pancreatitis: In search for a cuRE group are intended to bring standardization to the diagnosis and treatment of this rare childhood disorder. These statements may help guide a uniform approach to patient care and facilitate future research studies.

Autoimmune pancreatitis (AIP) is an autoimmune disorder of the pancreas characterized by enhanced IgG4 antibody responses and multiple organ involvement. AIP is a pancreatic manifestation of the systemic IgG4-related disease (IgG4-RD). Although AIP and IgG4-RD predominantly occur in middle-aged and elderly men, the roles of eating habits and lifestyle in the pathogenesis of these conditions are poorly understood. In this study, we examined whether a high-fat diet (HFD), preferred by middle-aged and elderly men, increases sensitivity to experimental AIP. We modeled AIP in MRL/MpJ mice by repeated injections of polyinosinic:polycytidylic acid. HFD exacerbated AIP development and promoted pancreatic accumulation of interferon (IFN)-α-producing plasmacytoid dendritic cells (pDCs). However, HFD did not increase the severity of autoimmune sialadenitis, another disorder associated with AIP and IgG4-RD. Neutralization of type I IFN signaling pathways prevented the development of severe AIP induced by HFD. In contrast, leaky gut was less likely to be associated with the HFD-induced exacerbation of AIP, as was evidenced by the lack of significant alterations in the jejunal or ileal expression of tight junction proteins. These data suggest that HFD exacerbates experimental AIP through the activation of pDCs producing IFN-α. • High-fat diet exacerbates experimental autoimmune pancreatitis (AIP). • Type I IFNs exacerbate AIP induced by high-fat diet. • Impaired function of tight junction proteins is not associated with AIP exacerbation. [ABSTRACT FROM AUTHOR]

Pancreatic cancer is a highly lethal disease associated with significant morbidity and mortality. In the United States (US), the overall 5-year relative survival rate for pancreatic cancer during the 2012–2018 period was 11.5%. However, the cancer stage at diagnosis strongly influences relative survival in these patients. Per the National Cancer Institute (NCI) statistics for 2012–2018, the 5-year relative survival rate for patients with localized disease was 43.9%, while it was 3.1% for patients with distant metastasis. The poor survival rates are primarily due to the late development of clinical signs and symptoms. Hence, early diagnosis is critical in improving treatment outcomes. In recent years, artificial intelligence (AI) has gained immense popularity in gastroenterology. AI-assisted endoscopic ultrasound (EUS) models have been touted as a breakthrough in the early detection of pancreatic cancer. These models may also accurately differentiate pancreatic cancer from chronic pancreatitis and autoimmune pancreatitis, which mimics pancreatic cancer on radiological imaging. In this review, we detail the application of AI-assisted EUS models for pancreatic cancer detection. We also highlight the utility of AI-assisted EUS models in differentiating pancreatic cancer from radiological mimickers. Furthermore, we discuss the current limitations and future applications of AI technology in EUS for pancreatic cancers. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) and IgG4-related disease (IgG4-RD) are new disease entities characterized by enhanced IgG4 antibody responses and involvement of multiple organs, including the pancreas and salivary glands. Although the immunopathogenesis of AIP and IgG4-RD is poorly understood, we previously reported that intestinal dysbiosis mediates experimental AIP through the activation of IFN-α- and IL-33-producing plasmacytoid dendritic cells (pDCs). Because intestinal dysbiosis is linked to intestinal barrier dysfunction, we explored whether the latter affects the development of AIP and autoimmune sialadenitis in MRL/MpJ mice treated with repeated injections of polyinosinic–polycytidylic acid [poly (I:C)]. Epithelial barrier disruption was induced by the administration of dextran sodium sulfate (DSS) in the drinking water. Mice co-treated with poly (I:C) and DSS, but not those treated with either agent alone, developed severe AIP, but not autoimmune sialadenitis, which was accompanied by the increased accumulation of IFN-α- and IL-33-producing pDCs. Sequencing of 16S ribosomal RNA revealed that Staphylococcus sciuri translocation from the gut to the pancreas was preferentially observed in mice with severe AIP co-treated with DSS and poly (I:C). The degree of experimental AIP, but not of autoimmune sialadenitis, was greater in germ-free mice mono-colonized with S. sciuri and treated with poly (I:C) than in germ-free mice treated with poly (I:C) alone, which was accompanied by the increased accumulation of IFN-α- and IL-33-producing pDCs. Taken together, these data suggest that intestinal barrier dysfunction exacerbates AIP through the activation of pDCs and translocation of S. sciuri into the pancreas. [ABSTRACT FROM AUTHOR]

Purpose: To develop a support vector machine (SVM) classifier using CT texture-based analysis in differentiating focal-type autoimmune pancreatitis (AIP) and pancreatic duct carcinoma (PD), and to assess the radiologists' diagnostic performance with or without SVM. Materials and methods: This retrospective study included 50 patients (20 patients with focal-type AIP and 30 patients with PD) who underwent dynamic contrast-enhanced CT. Sixty-two CT texture-based features were extracted from 2D images of the arterial and portal phase CTs. We conducted data compression and feature selections using principal component analysis (PCA) and produced the SVM classifier. Four readers participated in this observer performance study and the statistical significance of differences with and without the SVM was assessed by receiver operating characteristic (ROC) analysis. Results: The SVM performance indicated a high performance in differentiating focal-type AIP and PD (AUC = 0.920). The AUC for all 4 readers increased significantly from 0.827 to 0.911 when using the SVM outputs (p = 0.010). The AUC for inexperienced readers increased significantly from 0.781 to 0.905 when using the SVM outputs (p = 0.310). The AUC for experienced readers increased from 0.875 to 0.912 when using the SVM outputs, however, there was no significant difference (p = 0.018). Conclusion: The use of SVM classifier using CT texture-based features improved the diagnostic performance for differentiating focal-type AIP and PD on CT. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is a fibroinflammatory disease of the pancreas. Type 1 AIP is the pancreatic manifestation of a systemic IgG4-related disease and is associated with serum elevation of IgG4, tissue infiltration of IgG4-positive plasma cells, and multiorgan involvement. Although serum IgG4 elevation is considered a useful diagnostic tool, the concomitant presence of more diagnostic criteria is needed to achieve diagnosis. No other biomarkers have been approved in clinical practice in type 1 AIP. Type 2 AIP is a pancreatic-specific disease associated with inflammatory bowel disease. No specific biomarkers for type 2 AIP have been identified. The role of serum IgG4 in the diagnosis, management and follow-up of patients with type 1 AIP. Moreover, data on other emerging biomarkers for type 1 and 2 AIP have been reported. The diagnosis of AIP is challenging in clinical practice, especially for focal forms without multiorgan involvement, where distinction from pancreatic cancer can be difficult. Despite the strong association with type 1 AIP, serum IgG4 should only be measured when the suspicion for the disease is high, considering its limited sensitivity. New biomarkers with high diagnostic yield for both type 1 and type 2 AIP are needed. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is a rare disease. The diagnosis of AIP is difficult and should be made by a comprehensive evaluation of clinical, radiological, serological, and pathological findings. Two different types of AIP have been identified: autoimmune pancreatitis type 1 (AIP-1), which is considered a pancreatic manifestation of multiorgan disease related to IgG4, and autoimmune pancreatitis type 2 (AIP-2), which is considered a pancreas-specific disease not related to IgG4. Although the pathophysiological conditions seem to differ between type 1 and type 2 pancreatitis, both respond well to steroid medications. In this review, we focused on the pathogenesis of the disease to develop a tool that could facilitate diagnosis and lead to the discovery of new therapeutic strategies to combat autoimmune pancreatitis and its relapses. The standard therapy for AIP is oral administration of corticosteroids. Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. In selected patients, immunomodulators such as azathioprine are used to maintain remission. The strength of this review, compared with previous studies, is that it focuses on the clear difference between the two types of autoimmune pancreatitis with a clearly delineated and separate pathogenesis. In addition, the review also considers various therapeutic options, including biologic drugs, such as anti-tumor necrosis factor (TNF) therapy, a well-tolerated and effective second-line therapy for AIP type 2 relapses or steroid dependence. Other biologic therapies are also being explored that could provide a useful therapeutic alternative to corticosteroids and immunosuppressants, which are poorly tolerated due to significant side effects. [ABSTRACT FROM AUTHOR]

In 1995, Yoshida et al. proposed first the concept of "autoimmune pancreatitis" (AIP). Since then, AIP has been accepted as a new pancreatic inflammatory disease and is now divided two subtypes. Type 1 AIP affected immunoglobulin G4 (IgG4) and implicates the pancreatic manifestation of IgG4-related disease, while type 2 is characterized by neutrophil infiltration and granulocytic epithelial lesions (GEL). Recent research has clarified the clinical and pathophysiological aspects of type 1 AIP, which is more than type 2 among the Japanese population. However, many details remain unclear about the pathogenesis and progression of this disease. In this review, we discuss the current knowledge and recent advances relating to type 1 AIP. [ABSTRACT FROM AUTHOR]

Type 1 autoimmune pancreatitis (AIP) is a pancreatic manifestation of IgG4-related disease (IgG4-RD) and is a unique chronic inflammatory disease characterized by fibrosis. IgG4-RD is caused by an autoimmune mechanism that mimics malignant tumors and inflammatory disorders. Apoptosis inhibitor of macrophages (AIM) can function as a biomarker of autoimmune or inflammatory diseases with tissue fibrosis, including in inflammatory bowel disease and chronic liver disease. Therefore, the aim of the present study was to clarify the role of serum AIM levels and the clinical characteristics of patients with IgG4-RD and AIP. For this purpose, serum AIM concentrations were assessed using ELISA and the association between AIM and the laboratory and clinical data from patients with IgG4-RD/AIP, patients with pancreatic cancer (PC) and healthy controls (HCs), was determined. The results demonstrated that the serum AIM concentrations were not associated with the laboratory data. However, the serum AIM levels were significantly elevated in patients with AIP compared with the HCs and patients with PC. Furthermore, the serum AIM levels significantly decreased following steroid therapy in patients with AIP who were in remission. Overall, the present study demonstrates that serum AIM levels may be a potentially useful biomarker for the differential diagnosis of AIP and for evaluating the therapeutic reactivity of affected patients. [ABSTRACT FROM AUTHOR]

Objectives: To identify reliable MRI features for differentiating autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to summarize their diagnostic accuracy. Methods: We conducted a systematic literature review and meta-analysis using PubMed, EMBASE, and the Cochrane Library to identify original articles published between January 2006 and July 2021. The pooled diagnostic accuracy, including the diagnostic odds ratios (DORs) with 95% confidence intervals (CIs) of the identified features, was calculated using a bivariate random effects model. Results: Twelve studies were included, and 92 overlapping descriptors were subsumed under 16 MRI features. Ten features favoring AIP were diffuse enlargement (DOR, 75; 95% CI, 9–594), capsule-like rim (DOR, 52; 95% CI, 20–131), multiple main pancreatic duct (MPD) strictures (DOR, 47; 95% CI, 17–129), homogeneous delayed enhancement (DOR, 46; 95% CI, 21–104), low apparent diffusion coefficient value (DOR, 30), speckled enhancement (DOR, 30), multiple pancreatic masses (DOR, 29), tapered narrowing of MPD (DOR, 15), penetrating duct sign (DOR, 14), and delayed enhancement (DOR, 13). Six features favoring PDAC were target type enhancement (DOR, 41; 95% CI, 11–158), discrete pancreatic mass (DOR, 35; 95% CI, 15–80), upstream MPD dilatation (DOR, 13), peripancreatic fat infiltration (DOR, 10), upstream parenchymal atrophy (DOR, 5), and vascular involvement (DOR, 3). Conclusion: This study identified 16 informative MRI features to differentiate AIP from PDAC. Among them, diffuse enlargement, capsule-like rim, multiple MPD strictures, and homogeneous delayed enhancement favored AIP with the highest DORs, whereas discrete mass and target type enhancement favored PDAC. Key Points: • The MRI features with the highest pooled diagnostic odds ratios (DORs) for autoimmune pancreatitis were diffuse enlargement of the pancreas (75), capsule-like rim (52), multiple strictures of the main pancreatic duct (47), and homogeneous delayed enhancement (46). • The MRI features with the highest pooled DORs for pancreatic ductal adenocarcinoma were target type enhancement (41) and discrete pancreatic mass (35). [ABSTRACT FROM AUTHOR]

Rationale and Objectives: To determine the prevalence of diffuse involvement of pancreas and to identify the findings of malignancies using enhancement computed tomography (CT).Materials and Methods: A total of 1,0249 patients performed enhancement CT in our hospital over 62 months were investigated and the final study cohort includes 245 patients (170 males, 75 females; mean age, 56.94 ± 12.17 years). The reference standard is the final clinical/pathological diagnosis. The lesion-to-aorta enhancement ratio (LAR) on the pancreatic arterial phase, portal phase and delayed phase (DP) and the traditional CT findings were evaluated. Intergroup comparisons between malignancies and non-malignancies lesions were performed. Univariate and multivariate analyses were conducted to identify findings predicting malignancies.Results: The prevalence of malignancy was 45.3% (111/245) of diffuse enlargement of pancreas. All benign lesions were autoimmune pancreatitis 54.7% (n = 134). The most common malignant lesion was pancreatic ductal adenocarcinoma (n = 88, 35.9%). Other rare lesions with malignant potential included pancreatic neuroendocrine tumor (n = 11, 4.5%), lymphoma (n = 4, 1.6%), metastasis (n = 4, 1.6%), solid pseudopapillary neoplasm (n = 3, 1.2%) and acinar cell carcinoma (n = 1, 0.4%). Residual normal pancreas parenchyma, heterogeneity, short axis (cut-off value, 3.15 cm) and LARDP (cut-off value, 0.75) were independent predictors of malignancies. When the above predictors were combined, a sensitivity of 94.2%, a specificity of 90.8% were attained.Conclusion: Diffuse involvement of the pancreas is rare and is not a specific sign of autoimmune pancreatitis, and it is associated with a wide spectrum of malignant conditions. Dynamic enhancement CT is helpful to identifying malignancies. [ABSTRACT FROM AUTHOR]

Endoscopic ultrasound (EUS) provides high-resolution images of both pancreatic parenchyma and duct and therefore is an integral component of evaluating and treating patients with pancreatitis and its complications. The development of enhanced EUS imaging techniques and newer EUS-specific accessories has expanded the diagnostic and therapeutic role of EUS in patients with acute and chronic pancreatitis (CP). This review discusses the current diagnostic and therapeutic role of EUS in acute pancreatitis (AP), CP, and autoimmune pancreatitis (AIP). EUS plays a vital role in patients with AP by confirming the presence of common bile duct (CBD) stones in patients with acute biliary pancreatitis and intermediate probability of CBD stones. It plays an important role in the etiological evaluation of patients with idiopathic acute and recurrent pancreatitis. EUS is also an essential modality for diagnosing and managing pancreatico-biliary as well as gastroduodenal complications associated with CP. EUS-guided FNB using newer generation core biopsy needles has made possible accurate diagnosis of AIP by providing tissue samples with preserved architecture. [ABSTRACT FROM AUTHOR]

We herein report a case of IgG4-related autoimmune hepatitis (AIH) in a patient with a history of type 1 autoimmune pancreatitis. A 56-year-old man presented with fatigue and jaundice at our hospital. A blood biochemistry analysis revealed significant liver dysfunction, positive results for antinuclear antibodies, and high serum IgG4 levels. A histopathological examination revealed interface hepatitis marked by IgG4-positive plasma cell infiltration in the portal area, leading to liver cell depletion and necrosis. Based on the diagnosis of IgG4-related AIH, prednisolone treatment was initiated, which led to the rapid resolution of liver dysfunction and jaundice. An accurate diagnosis of IgG4-related AIH is crucial to prevent secondary manifestations.

A 50-year-old man was diagnosed with type 1 autoimmune pancreatitis (AIP) following endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and a histopathological examination. After six months of untreated follow-up, the serum IgG4 level decreased, and the diffuse pancreatic enlargement improved; however, a pancreatic head mass became apparent. EUS-FNA of this mass revealed pancreatic ductal adenocarcinoma (PDAC) with IgG4-positive plasma cells. In addition, the resected specimen revealed PDAC, without any features of AIP. After pancreatoduodenectomy, AIP did not recur. The development of AIP in this case could be related to paraneoplastic syndrome.

Abstract Background Autoimmune pancreatitis (AIP) is a rare disease that manifests as pancreatic involvement in systemic IgG4-related disease (IgG4-RD), a special type of chronic pancreatitis caused by autoimmune abnormalities. The main imaging manifestations of IgG4-related AIP consist of diffuse or localized pancreatic enlargement and irregular pancreatic duct narrowing. The diagnosis of AIP is challenging because it can present with focal lesions, similar to radiologically bile duct cancer or pancreatic cancer. Case presentation A 55-year-old male patient was admitted with painless jaundice and multiple radiographic findings of pancreatic head mass, as well as intrahepatic and extrahepatic bile duct dilatation. Various imaging methods indicated pancreatic cancer. However, the endoscopic ultrasonography guided fine needle aspiration (EUS-FNA) and a laparoscopic pancreatic biopsy suggested an IgG4-related AIP. After one month, magnetic resonance imaging showed that the lesion had slightly grown. Combined with CA19-9 and other indexes, the possibility of malignancy was high and there were still surgical indications. The pathological analysis following a pancreaticoduodenectomy revealed poorly differentiated adenocarcinoma in the distal common bile duct. Conclusion To date, few reports have described pancreatic or extrapancreatic malignancies in AIP patients, and no association between AIP and bile duct adenocarcinoma has been previously confirmed. This case discuss the differentiation between AIP and malignancy, recent research progress, and the correlation between the two diseases, highlights the importance of carefully evaluating patients with AIP to rule out potential tumors, as well as the critical need for follow up treatment.

Abstract Purpose To assess the value of the multimodel magnetic resonance imaging (MRI), including unenhanced images, dynamic contrast-enhanced MRI (DCE-MRI), MR-cholangiopancreatography (MRCP), and diffusion-weighted imaging (DWI), in differentiation of mass-forming autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC). Methods Twelve patients with mass-forming AIP and 30 with PDAC were included. All patients underwent unenhanced MRI, DCE-MRI, DWI, and MRCP. Relevant values including sensitivity and specificity of the imaging features and their diagnostic performance for predicting mass-forming AIP were analyzed. Results Several statistically significant MR findings and quantitative indexes differentiating mass-forming AIP from PDAC, including multiplicity, irregularity or conformation, capsule-like rim enhancement, absence of internal cystic or necrotic portion, homogeneous enhancement during pancreatic, venous, and delayed phases, skipped stricture or stricture of MPD, absence of side branch dilation, maximum upstream MPD diameter 0.739, ContrastAP > 0.710, ContrastPP > 0.879, and ContrastVP or ContrastDP > 0.949, indicated mass-forming AIP (P

Aims/Introduction: To investigate factors influencing glycemic control in diabetes mellitus complicated by autoimmune pancreatitis. Materials and Methods: This retrospective cohort study investigated 33 patients with diabetes mellitus complicated by autoimmune pancreatitis who had received steroid therapy at Toranomon Hospital between January 1, 2011, and December 31, 2020. The course of glycemic control at 12 months after starting steroids was classified into three groups: Improved, Unchanged, or Worsened. Factors affecting these groups were investigated. Furthermore, we created two scores: (1) time of diabetes mellitus onset and baseline body mass index; (2) time of diabetes mellitus onset and baseline C‐peptide index. Diabetes mellitus occurring at the same time as autoimmune pancreatitis, body mass index ≥22 kg/m2, and C‐peptide index ≥1.1 were each worth 1 point. Scores were summed and totals of 0–2 were compared between groups. Results: Ten patients were in the Improved group, 10 were in the Unchanged group, and 13 were in the Worsened group. The baseline body mass index and baseline C‐peptide index were lower in the Worsened group than in the Improved group (P < 0.05 each). In addition, the scores were lower in the Worsened group than in the other groups (P < 0.05). Conclusions: Patients with a lower baseline body mass index and a decreased baseline C‐peptide index may experience worse glycemic control on steroid therapy. [ABSTRACT FROM AUTHOR]

Background and Aim: Autoimmune pancreatitis (AIP) and inflammatory bowel disease (IBD) are categorized into immune‐mediated inflammatory disorders (IMIDs). While AIP is a pancreato‐biliary IMID with an increased incidence and prevalence among patients with IBD, its features are still unclear. This systematic review and meta‐analysis aims to assess the prevalence and clinical characteristics of AIP‐IBD patients. Methods: Electronic databases were searched to identify observational studies assessing AIP and IBD. The primary outcome was the prevalence of IBD among AIP patients, and vice versa. Secondary outcomes included clinical findings and outcomes of each IMID in AIP‐IBD patients. The pooled rate of each outcome was determined using a random effects model. Results: For primary outcomes, 40 observational studies with 4031 AIP patients were included and the pooled prevalence of IBD was 10.5% (95% CI 7.2–15.0%). Meanwhile, five studies with 10,551 IBD patients were included and the pooled prevalence of AIP was 0.6% (95% CI 0.2–1.9%). For secondary outcomes, 53 observational studies with 469 AIP‐IBD patients were assessed. The rates of type 2 AIP and ulcerative colitis were 79.2% (95% CI 69.1–86.6%) and 74.8% (95% CI 68.2–80.4%), respectively. We also demonstrated AIP‐IBD patients were at a significant increased risk of AIP recurrence and colectomy compared with patients with either AIP or IBD (RR = 1.9, 95% CI 1.1–3.1 and P = 0.014 and RR = 3.7, 95% CI 1.9–6.9, P < 0.001, respectively). Conclusions: Our meta‐analysis reported the prevalence of AIP‐IBD patients and demonstrated patients with both IMIDs had a high risk of poor outcomes. [ABSTRACT FROM AUTHOR]

The diagnosis of autoimmune pancreatitis (AIP) and immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) may require a somewhat invasive pathological examination and steroid responsiveness. This retrospective study assessed the complemental diagnosis of AIP and IgG4-SC using submandibular gland (SG) ultrasonography (US) in 69 patients, including 54 patients with AIP, 2 patients with IgG4-SC, and 13 patients with both AIP and IgG4-SC. The data from the physical examination and US of SGs to diagnose AIP (n = 67) and IgG4-SC (n = 15) were analyzed. The steroid therapy efficacy in resolving hypoechoic lesions in SGs was evaluated in 36 cases. The presence of IgG4-related pancreaticobiliary disease with multiple hypoechoic lesions in SGs was reduced from 31 to 11 cases after steroid therapy, suggesting that multiple hypoechoic lesions in SGs are strongly associated with IgG4-positive cell infiltrations. Multiple hypoechoic lesions in SGs were observed in 53 cases, whereas submandibular swelling on palpation was observed in 21 cases of IgG4-related pancreaticobiliary diseases. A complemental diagnosis of IgG4-related pancreaticobiliary diseases without a histological diagnosis and steroid therapy was achieved in 57 and 68 cases without and with multiple hypoechoic lesions in SGs, respectively. In conclusion, multiple hypoechoic lesions in SGs are useful for the complemental diagnosis of IgG4-related pancreaticobiliary diseases. [ABSTRACT FROM AUTHOR]

Background: There is conflicting evidence regarding autoimmune pancreatitis (AIP) association with pancreatic and non-pancreatic cancers. Literature lacks data on overall prevalence of malignancies in autoimmune pancreatitis. Aim: Given the lack of definite evidence, we aimed to pool and summarize data from available literature regarding prevalence of different malignancies in AIP. Methods: We conducted a systematic search of MEDLINE, EMBASE, Cochrane Register of Controlled Trials, and Web of Science through February 16, 2021, to include observational studies assessing the incidence of cancer in AIP. We used the DerSimonian–Laird method with random effects for meta-analysis. Pooled prevalence, 95% confidence interval (CI), and I2 statistic are reported. Results: A total of 17 studies with 2746 patients were included assessing the prevalence of cancer in AIP. The overall prevalence of cancer in AIP was 9.6% [95% confidence interval (CI), 5.7–13.5%]. The cancers with the highest prevalence in AIP population were gastric and colorectal cancer, with prevalence of 1.3% (95% CI, 0.5–2.1%) and 1.2% (95% CI, 0.6–1.8%), respectively. Conclusion: We demonstrate the prevalence of different cancers in AIP. Inflammatory surge in AIP and subsequent carcinogenesis is one explanation for this association. Moreover, AIP can be a paraneoplastic syndrome manifestation of malignancies. [ABSTRACT FROM AUTHOR]

Background: Autoimmune pancreatitis (AIP) is a specific form of chronic pancreatitis with a high relapse rate after treatment. AIP patients are burdened with an increased risk of long-term sequelae such as exocrine and endocrine insufficiency. Our objective was to investigate if pharmacological treatment affects both endocrine and exocrine pancreatic function in patients with AIP. Methods: We included 59 patients with definite AIP in the final analysis. Screening for diabetes mellitus (DM) and pancreatic exocrine insufficiency (PEI) was performed at the time of AIP diagnosis and during follow-up. Results: There were 40 (67.8%) males and 19 (32.2%) females; median age at diagnosis was 65 years. Median follow-up after the diagnosis of AIP was 62 months. PEI prevalence at diagnosis was 72.7% and was 63.5% at follow-up. The cumulative incidence of DM was 17.9%, with a prevalence of DM at diagnosis of 32.8%. No strong association was found between pharmacological treatment and occurrence of PEI and DM. Univariate analysis identified potential risk factors for PEI (other organ involvement and biliary stenting) and for DM (overweight, blue-collar profession, smoking, weight loss or obstructive jaundice as presenting symptoms, imaging showing diffuse pancreatic enlargement, smoking). In a multivariate analysis, only obstructive jaundice was identified as a risk factor for DM both at diagnosis and during follow-up. Conclusions: Our results suggest that the prevalence of endocrine and exocrine insufficiency in AIP is high at diagnosis with an additional risk of PEI and DM during follow-up despite pharmacological treatment. [ABSTRACT FROM AUTHOR]

The accurate differential diagnosis between autoimmune pancreatitis (AIP) and pancreatic ductal adenocarcinoma (PDAC) is clinically important. We aimed to determine significant MRI features for differentiating AIP from PDAC, including assessment of diffusion-weighted imaging (DWI). We performed a systematic search using three databases. The pooled diagnostic odds ratio was calculated using a bivariate random effects model to determine significant MRI features for differentiating AIP from PDAC. The pooled sensitivity and specificity were calculated. The qualitative systematic review for DWI assessment was performed. Of nine studies (775 patients), multiple main pancreatic duct (MPD) strictures, absence of upstream marked MPD dilatation, peripancreatic rim, and duct penetration sign were significant MRI features for differentiating AIP from PDAC. Absence of MPD dilatation had the highest pooled sensitivity (87%, 95% CI=68–96%), whereas peripancreatic rim had the highest pooled specificity (100%, 95% CI=88–100%). Of 12 studies evaluating DWI, seven reported statistically significant differences in apparent diffusion coefficient (ADC) values between AIP and PDAC; however, four reported lower ADC values in AIP than in PDAC, but three reported the opposite result. The four significant MRI features can be useful to differentiate AIP from PDAC, but DWI assessment might be limited. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is a rare etiological type of chronic pancreatitis. The clinical and radiological presentation of AIP often resembles that of pancreatic cancer. Identifying non-invasive markers for their early distinction is of utmost importance to avoid unnecessary surgery or a delay in steroid therapy. Thus, this systematic review was conducted to revisit all current evidence on the clinical utility of different serum biomarkers in diagnosing AIP, distinguishing AIP from pancreatic cancer, and predicting disease course, steroid therapy response, and relapse. A systematic review was performed for articles published up to August 2021 by searching electronic databases such as MEDLINE, Web of Science, and EMBASE. Among 5123 identified records, 92 studies were included in the qualitative synthesis. Apart from immunoglobulin (Ig) G4, which was by far the most studied biomarker, we identified autoantibodies against the following: lactoferrin, carboanhydrase II, plasminogen-binding protein, amylase-α2A, cationic (PRSS1) and anionic (PRSS2) trypsinogens, pancreatic secretory trypsin inhibitor (PSTI/SPINK1), and type IV collagen. The identified novel autoantigens were laminin 511, annexin A11, HSP-10, and prohibitin. Other biomarkers included cytokines, decreased complement levels, circulating immune complexes, N-glycan profile changes, aberrant miRNAs expression, decreased IgA and IgM levels, increased IgE levels and/or peripheral eosinophil count, and changes in apolipoprotein isoforms levels. To our knowledge, this is the first systematic review that addresses biomarkers in AIP. Evolving research has recognized numerous biomarkers that could help elucidate the pathophysiological mechanisms of AIP, bringing us closer to AIP diagnosis and its preoperative distinction from pancreatic cancer. [ABSTRACT FROM AUTHOR]

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