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Objective Steroid pulse therapy is a regimen involving the intravenous administration of supra-pharmacological doses of corticosteroids in the short term. It is used to treat various inflammatory and autoimmune conditions. However, the strengths and limitations of steroid pulse therapy for induction of remission in type 1 autoimmune pancreatitis (AIP) are unknown. Methods Depending on the steroid therapy regimen administered, the 104 patients with type 1 AIP included in this retrospective study were divided into three groups: conventional oral prednisolone (PSL) regimen (PSL group), intravenous methylprednisolone (IVMP) pulse followed by oral PSL regimen (Pulse+PSL group), and IVMP pulse-alone regimen (Pulse-alone group). We then examined the relapse rate and adverse events among the three groups. Results The Kaplan-Meier estimates of the relapse rate at 36 months after steroid therapy were 13.6% in the PSL group, 13.3% in the Pulse+PSL group, and 46.2% in the Pulse-alone group. The log-rank test revealed that the relapse-free survival in the Pulse-alone group was significantly shorter than that in the PSL (p=0.024) and Pulse+PSL groups (p=0.014). The exacerbation of glucose tolerance after steroid therapy was less frequently observed in the Pulse-alone group (0%) than in the PSL group (17%, p=0.050) and Pulse+PSL groups (26%, p=0.011). Conclusion Although treatment with IVMP pulse alone resulted in unsatisfactory relapse prevention outcomes compared with conventional steroid therapy, the IVMP pulse-alone regimen might be an alternative treatment strategy for type 1 AIP from the perspective of avoiding adverse events from steroids.

Self-expandable metallic stents (SEMSs) are widely used for malignant biliary stricture (MBS). Acute pancreatitis is an early complication following SEMS placement. In the present case, the patient developed severe acute pancreatitis after SEMS placement for MBS because of metastatic lymph nodes. Endoscopic retrograde cholangiopancreatography, endoscopic sphincterotomy and an endoscopic nasobiliary drainage tube placement were performed. After seven days, an uncovered SEMS was placed; however, severe acute pancreatitis occurred, and the SEMS was drawn out emergently. In SEMS placement for patients with MBS caused by non-pancreatic cancer, SEMS should be selected carefully while considering each patient's case.

The characteristic finding of sausage-shaped pancreas or capsule-like rim facilitates the diagnosis of autoimmune pancreatitis. We herein report a case of a 67-year-old man showing a sausage-shaped, enlarged pancreas with a capsule-like rim on computed tomography. Furthermore, endoscopic retrograde cholangiopancreatography demonstrated diffuse narrowing of the main pancreatic duct, in addition to stenosis of the lower bile duct. Finally, we were able to diagnose pancreatic cancer in this patient by an endoscopic ultrasound-guided fine-needle aspiration biopsy following peroral cholangioscopy and bile cytology. This report emphasizes the significance of pathological confirmation before starting treatment, even in cases with diffuse pancreatic enlargement.

Recent histological and clinical studies have suggested the existence of 2 distinct types of autoimmune pancreatitis (AIP): type 1 AIP related to IgG4, exhibiting lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP related to granulocyte epithelial lesions (GELs), exhibiting idiopathic duct-centric chronic pancreatitis (IDCP). We herein present a case of type 1 AIP with histologically proven LPSP with GELs. This patient had neither serum IgG4 elevation nor MPD narrowing. In this case, the clinically and histologically atypical findings for type 1 AIP are intriguing.

A 65-year-old woman with elevated serum levels of pancreatic enzymes was referred to our hospital for further examinations. Abdominal US and contrast-enhanced CT demonstrated swelling of the pancreas body and tail. MRCP and ERCP revealed abrupt ending of the MPD in the pancreas body. Under the suspicion of malignancy, distal pancreatectomy and splenectomy were performed. The histopathological findings showed idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesions (GEL). As most cases of Japanese autoimmune pancreatitis (AIP) are lymphoplasmacytic sclerosing pancreatitis (LPSP), the present case seems to be helpful to clarify the clinical findings of IDCP in Japan.

Xanthogranulomatous inflammation (XGI) is histopathologically characterized by a marked proliferative fibrosis, parenchymal destruction, and infiltration of foamy histiocytes intermixed with other inflammatory cells. Herein, we report a case of a 73-year-old man without symptoms who was initially diagnosed with a pancreatic cystic tumor but later with XGI in the peripancreatic region. Although XGI has been reported to occur in various organs or tissues, such as the gallbladder, kidney, bone, stomach, colon, appendix, lymph nodes, and soft tissues, XGI involving the pancreas or its surrounding tissues is extremely rare. When a pancreatic cystic lesion does not have typical clinicoradiological features of common pancreatic cystic neoplasms, this pathologic condition should be considered in the differential diagnosis.

We report a very rare case of benign biliary stricture with calcification and porcelain gallbladder, causing difficulty in differential diagnosis. A 64-year-old man was referred for further examination of jaundice. Computed tomography showed calcifications in the gallbladder wall and the common bile duct. Endoscopic retrograde cholangiopancreatography revealed narrowing and a filling defect in the distal common bile duct. Peroral cholangioscopy showed a protruded lesion and stricture, and pathological examinations revealed no evidence of malignancy. The stricture was resolved after temporary insertion of progressively larger of plastic stents. Patients with benign biliary stricture and/or porcelain gallbladder should be followed carefully, because malignancy can occur as a complication, although infrequent.

Recently, it has been reported that autoimmune pancreatitis (AIP) can be complicated with various extrapancreatic lesions. Here, we report a very rare case of pancreatic and hepatic inflammatory pseudotumor (IPT) with the infiltration of IgG4-positive plasmacytes. The patient showed pancreatic and hepatic masses with elevated levels of serum IgG4. Endoscopic retrograde cholangiopancreatography revealed narrowing of the intrapancreatic bile duct. Fluorine-18fluorodeoxyglucose positron emission tomography suggested pancreatic cancer with hepatic metastasis. Histopathologic findings showed fibrosis and infiltration of IgG4-positive plasmacytes, suggesting IPT. The present case suggests a possible common mechanism in the development of AIP and IPT of the liver.