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Background The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort. Aims To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP. Methods We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP. Results AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI. Conclusions The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.

Background Chronic pancreatitis is a known risk factor of pancreatic cancer (PDAC). A similar association has been suggested but not demonstrated for autoimmune pancreatitis (AIP). Objective The aim of our study was to identify and analyse all published cases of AIP and PDAC co-occurrence, focusing on the interval between the diagnoses and the cancer site within the pancreas. Methods Relevant studies were identified through automatic searches of the MEDLINE, EMBASE, Scopus, and Web of Science databases, and supplemented by manual checks of reference lists in all retrieved articles. Missing/unpublished data were obtained from the authors of relevant publications in the form of pre-prepared questionnaires. Results A total of 45 cases of PDAC in AIP patients were identified, of which 12 were excluded from the analysis due to suspicions of duplicity or lack of sufficient data. Thirty-one patients (94%) had type 1 AIP. Synchronous occurrence of PDAC and AIP was reported in 11 patients (33%), metachronous in 22 patients (67%). In the metachronous group, the median period between diagnoses was 66.5 months (2–186) and a majority of cancers (86%) occurred more than two years after AIP diagnosis. In most patients (70%), the cancer originated in the part of the pancreas affected by AIP. Conclusions In the literature, there are reports on numerous cases of PDAC in AIP patients. PDAC is more frequent in AIP type 1 patients, typically metachronous in character, and generally found in the part of the pancreas affected by AIP.

Biliary strictures caused by inflammation or fibrosis lead to jaundice and cholangitis which often make it difficult to distinguish malignant strictures. In cases when malignancy cannot be excluded, surgery is often performed. The concept of immunoglobulin G4 (IgG4)-related sclerosing cholangitis (SC) as a benign biliary stricture was recently proposed. The high prevalence of the disease in Asian countries has resulted in multiple diagnostic and treatment guidelines; however, there is need to formulate a standardized diagnostic strategy among various countries considering the utility, invasiveness, and cost-effectiveness. We evaluated accuracies of various diagnostic modalities for biliary strictures comparing pathology in the Delphi meetings which were held in Rochester, MN. The diagnostic utility for each modality was graded according to the experts, including gastroenterologists, endoscopists, radiologists, and pathologists from the United States and Japan. Diagnostic utility of 10 modalities, including serum IgG4 level, noninvasive imaging, endoscopic ultrasound, endoscopic retrograde cholangiopancreatography-related diagnostic procedures were advocated and the reasons were specified. Serum IgG4 level, noninvasive imaging, diagnostic endoscopic ultrasound and intraductal ultrasonography under endoscopic retrograde cholangiopancreatography were recognized as useful modalities for the diagnosis. The information in this article will aid in the diagnosis of biliary strictures particularly for distinguishing IgG4-SC from cholangiocarcinoma and/or primary SC.

Background Extracorporeal shock wave lithotripsy is the first-line therapy for large pancreatic duct stones; however, it requires a long duration of therapy. Aims To clarify the effect of pancreatic stenting prior to extracorporeal shock wave lithotripsy on shortening the duration of therapy and reducing complications. Methods We retrospectively compared 45 patients who underwent pancreatic stenting prior to extracorporeal shock wave lithotripsy (stenting group) and 35 patients who did not undergo stenting prior to extracorporeal shock wave lithotripsy (non-stenting group) with regard to the cumulative number of shock waves required for stone fragmentation (stone size Results The stenting group was associated with a significantly lower cumulative number of shock waves in univariate analysis (log-rank, p = 0.046) and multivariate Cox proportional hazard analysis (hazard ratio, 1.88; 95% confidence interval, 1.13–3.14; p = 0.015) than the non-stenting group. The frequency of pancreatitis tends to be lower in the stenting group than the non-stenting group (2.2% [1/45] vs 11.4% [4/35]; p = 0.162). Conclusions Pancreatic stenting prior to extracorporeal shock wave lithotripsy reduced the cumulative number of shock waves required for pancreatic stone fragmentation, and could be useful to shorten the duration of therapy.

Background: Comprehensive immunostaining evaluation of the biopsy specimens from various organs with type 1 autoimmune pancreatitis (AIP) has not been elucidated. Our aim was to clarify which of these biopsy specimens and counting method could be a useful tool for supporting the diagnosis of AIP. Methods: We retrospectively evaluated biopsy specimens from pancreas (n ¼ 19), stomach (n ¼ 28), duodenum (n ¼ 27), duodenal papilla (n ¼ 25), colon (n ¼ 19), liver (n ¼ 11), bile duct (n ¼ 24), and minor salivary gland (n ¼ 13) in 36 patients with AIP. Positive IgG4 immunostaining (>10 plasma cells/highpower field [HPF]) and positive IgG4/IgG ratio (>40%) of biopsy specimens from 8 sites of 6 organs in one HPF and an average from 3 HPFs were compared between AIP and controls. Results: The sensitivity of IgG4 immunostaining for AIP in one HPF were 16% in pancreas,14% in stomach, 15% in duodenum, 52% in duodenal papilla, 11% in colon, 27% in liver, 21% in bile duct and 8% in minor salivary gland, respectively. The positive IgG4 immunostaining of the duodenal papilla in one HPF showed the highest sensitivity (52%) and accuracy (73%) among the 8 sites. It also showed the highest sensitivity among 4 different counting methods (IgG4 immunostaining in one HPF and 3 HPFs, both IgG4 immunostaining and IgG/IgG4 ratio in one HPF and 3 HPFs), but there were no significant differences with respect to specificity and accuracy. Conclusions: IgG4 immunostaining of swollen duodenal papilla with more than 10 IgG4-positive plasma cells in at least one HPF is useful for supporting the diagnosis of AIP.

Background and Aims Uncovered self-expandable metal stents (U-SEMSs) and covered self-expandable metal stents (C-SEMSs) are available for palliative therapy for malignant gastric outlet obstruction (GOO). However, clinical differences and indications between the 2 types of SEMSs have not been elucidated. Methods We retrospectively compared 126 patients with U-SEMS and 126 patients with C-SEMSs with regard to clinical outcome and factors predictive of clinical improvement after SEMSs placement. Results No significant difference was observed between the U-SEMS and C-SEMS groups with respect to technical success, clinical success, GOO score, or time to stent dysfunction. Stent migration was significantly more frequent in patients with C-SEMSs (U-SEMSs, .79%; C-SEMSs, 8.73%; P = .005). Karnofsky performance status, chemotherapy, peritoneal dissemination, and stent expansion ≤ 30% were associated significantly with poor GOO score improvement in multivariable analyses, but stent type was not ( P = .213). In subgroup analyses, insufficient (≤30%) stent expansion was an independent factor in patients with U-SEMSs ( P = .041) but not C-SEMSs. In the insufficient stent expansion subgroup, C-SEMSs was associated significantly with superior clinical improvement compared with U-SEMSs ( P = .01). Insufficient stent expansion was observed more frequently in patients with GI obstruction because of anastomotic sites or metastatic cancer (44.8% [13/29], P = .001). Conclusions No clinical difference, apart from stent migration, was observed between patients with U-SEMSs and C-SEMSs. GI obstruction because of an anastomotic site or metastatic cancer may be an indication for C-SEMS use to improve oral intake after SEMSs placement.