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Background and Aim: This study aimed to determine the clinical characteristics of immunoglobulin G4 (IgG4)-associated sclerosing cholangitis (ISC) and provide clinical clues differentiating ISC from primary sclerosing cholangitis (PSC) or hilar cholangiocar- cinoma (CCC). Methods: Sixteen patients with ISC manifesting as hilar/intrahepatic strictures were ana- lyzed for clinical characteristics and compared with patients with PSC and hilar CCC as disease controls for histology and serum IgG4 levels. Results: Distinguished biliary imaging findings of ISC included multifocal biliary tree involvement (n = 14), concentric bile duct thickening with preserved luminal patency (n = 13), and relatively mild proximal dilatation, despite prominent bile duct thickening (n = 11). Serum IgG4 levels were elevated in 12 patients (75%), but not in any of the 25 patients with hilar CCC.Ten patients (63%) had a past or concurrent history of autoimmune pancreatitis (AIP). The significant infiltration of IgG4-positive cells was observed with endobiliary or liver biopsy in 11 of 16 patients (69%) with ISC, but not in any patients with PSC or hilar CCC. Extrabiliary organ involvement, including sialadenitis, inflammatory pseudotumor of the liver and kidney, and retroperitoneal fibrosis, was present in seven patients. Marked improvement of biliary strictures and/or extrabiliary involvement was observed in all ISC patients after steroid therapy. Conclusions: ISC should be considered in the differential diagnosis of hilar/intrahepatic biliary strictures. Past or concurrent AIP or extrabiliary organ involvement strongly sug- gests the possibility of ISC. Significant infiltration of IgG4-positive cells on endobiliary or liver biopsy specimens, and/or elevated serum IgG4 levels, highly support the diagnosis of ISC and provide the rationale for steroid therapy.

Background Autoimmune pancreatitis (AIP) is now considered to be part of an immunoglobulin G4 (IgG4)-related systemic fibroinflammatory disease. Objective We evaluated whether IgG4 immunostaining of duodenal papillary biopsy specimens is useful for supporting a diagnosis of AIP. Design A prospective study. Setting A tertiary academic center. Patients/Interventions We obtained 2 forceps biopsy specimens from the major duodenal papilla (MDP) of 19 symptomatic AIP patients during ERCP before steroid administration. As a control, biopsy specimens were obtained from the MDP of patients with pancreatic cancer (n = 35), cholangiocarcinoma (n = 20), ampullary cancer (n = 11), ordinary chronic pancreatitis (n = 18), and AIP in remission (n = 16) and immunohistochemically examined. Main Outcome Measurements Specimens were considered positive for IgG4 immunostaining if there were more than 10 IgG4-positive plasma cells per high-power field. Results Positive IgG4 immunostaining of the MDP was found in 10 (53%) of 19 symptomatic AIP patients, but was absent in the control groups. Among symptomatic AIP patients (n = 19), 5 (83%) of 6 AIP patients with elevated serum IgG4 levels exhibited positive IgG4 staining of the MDP, whereas 5 (38%) of 13 AIP patients with normal serum IgG4 levels showed positive IgG4 staining of the MDP. Nine of 19 symptomatic AIP patients also underwent pancreatic biopsy, and positive IgG4 immunostaining of the MDP was observed in patients with positive pancreas IgG4 staining (4/6, 67%), but not in patients with negative pancreas IgG4 staining (0/3). Limitations Small symptomatic AIP patient population. Conclusions Positive IgG4 immunostaining of the MDP was an extremely specific and moderately sensitive tool for the diagnosis of AIP. IgG4 immunostaining of the MDP may be useful for supporting a diagnosis of AIP, especially when AIP is suspected clinically but serum IgG4 levels are normal or pancreatic tissue is not available.

Background Percutaneous transhepatic biliary drainage (PTBD) may be the last resort for an occluded biliary metal stent when the ERCP was unsuccessful. Objective Because an EUS-guided biliary drainage has been proposed as an effective alternative for PTBD after a failed ERCP, we conducted this study to determine the feasibility and usefulness of an EUS-guided hepaticogastrostomy (EUS-HG) with a fully covered self-expandable metal stent (FCSEMS) for an occluded biliary metal stent after a failed ERCP. Design A case study. Setting A tertiary referral center. Patients and Interventions Five patients who had an occluded biliary metal stent inserted after a hilar bilateral metal stent or a combined duodenal and biliary metal stent insertion and for whom reinterventional ERCP was unsuccessful underwent an EUS-HG with an FCSEMS for alternative PTBD. Main Outcome Measurements Technical and functional success, procedural complications, reinterventional rate after EUS-HG with an FCSEMS, and short-term stent patency. Results In all 5 patients, an EUS-HG with an FCSEMS was technically successful. No procedural complications, such as bile peritonitis, cholangitis, and pneumoperitoneum, were observed. Functional success was also 100% (5/5). During the follow-up period (median 152 days, range 64-184 days), no late complications, such as stent migration and occlusion, were observed. Thus, no biliary reintervention was performed during the follow-up period. Limitations A small series of patients without a control group. Conclusions The EUS-HG with an FCSEMS may be feasible, effective, and an alternative PTBD for an occluded biliary metal stent after a failed ERCP.

Background/Aims: We aimed to evaluate survival time and prognostic factors in patients with advanced unresectable cholangiocarcinoma who have not re- ceived surgery, chemotherapy, or radiotherapy. Me- thods: A total of 1,377 patients, who were diagnosed with primary cholangiocarcinoma between 1996 and 2002, were reviewed retrospectively according to the following inclusion criteria: histologically proven pri- mary adenocarcinoma arising from the bile-duct epi- thelium, advanced unresectable stages, no severe co- morbidity that can affect survival time, and no history of surgery, chemotherapy, or radiotherapy. Results: Of the 1,377 cases reviewed, 330 patients complied with the inclusion criteria and were thus eligible to participate in this study; 203 had intrahepatic chol- angiocarcinoma and 127 had hilar cholangiocarcinoma. The overall survival time of the entire cohort (n=330) was median 3.9 months (range; 0.2 to 67.1). The sur- vival time was significantly shorter in the intrahepatic cholangiocarcinoma group (3.0±5.3 months) than in the hilar cholangiocarcinoma group (5.9±10.1 months; Kaplan-Meier survival analysis). Multivariate analysis revealed that distant metastasis was a poor prog- nostic factor for intrahepatic cholangiocarcinoma (p 3.0 g/dL was a fa- vorable prognostic factor (p=0.02), and baseline serum carcinoembryonic antigen level >30 ng/mL was a poor prognostic factor for hilar cholangiocarcinoma (p=0.01). Conclusions: The median survival of ad- vanced unresectable cholangiocarcinoma is dismal. (Gut and Liver 2009;3:298-305)

This study aimed to determine the clinical characteristics of immunoglobulin G4 (IgG4)-associated sclerosing cholangitis (ISC) and provide clinical clues differentiating ISC from primary sclerosing cholangitis (PSC) or hilar cholangiocarcinoma (CCC).Sixteen patients with ISC manifesting as hilar/intrahepatic strictures were analyzed for clinical characteristics and compared with patients with PSC and hilar CCC as disease controls for histology and serum IgG4 levels.Distinguished biliary imaging findings of ISC included multifocal biliary tree involvement (n = 14), concentric bile duct thickening with preserved luminal patency (n = 13), and relatively mild proximal dilatation, despite prominent bile duct thickening (n = 11). Serum IgG4 levels were elevated in 12 patients (75%), but not in any of the 25 patients with hilar CCC. Ten patients (63%) had a past or concurrent history of autoimmune pancreatitis (AIP). The significant infiltration of IgG4-positive cells was observed with endobiliary or liver biopsy in 11 of 16 patients (69%) with ISC, but not in any patients with PSC or hilar CCC. Extrabiliary organ involvement, including sialadenitis, inflammatory pseudotumor of the liver and kidney, and retroperitoneal fibrosis, was present in seven patients. Marked improvement of biliary strictures and/or extrabiliary involvement was observed in all ISC patients after steroid therapy.ISC should be considered in the differential diagnosis of hilar/intrahepatic biliary strictures. Past or concurrent AIP or extrabiliary organ involvement strongly suggests the possibility of ISC. Significant infiltration of IgG4-positive cells on endobiliary or liver biopsy specimens, and/or elevated serum IgG4 levels, highly support the diagnosis of ISC and provide the rationale for steroid therapy.

Background/Aims: With the progress of product development, single-step endoscopic ultrasound (EUS)guided transmural drainage can overcome some disadvantages of the blind or two-step procedures used in the treatment of pancreatic pseudocysts. We therefore evaluated the technical feasibility, efficacy, and safety of single-step EUS-guided transmural drainage of pancreatic pseudocysts. Methods: Endoscopic drainage of pancreatic pseudocysts was performed in 47 patients (median age, 46 years; range, 38 years to 59 years; 40 men) by using interventional echoendoscopes with a single-step device suitable for ballooning, bougination, and plastic-stent insertion. Results: Endoscopic stent placement was successful in 42 patients (89%; transgastric approach, 34/38; transduodenal approach, 8/9) and failed in 5 patients because of acute angulation (n=4) or small cyst (n=1). The volume of the pseudocyst was reduced by more than 90% or it disappeared completely in all of 41 patients (100%), based on a mean follow-up period of 17 months (range, 11 months to 20 months). The overall recurrence rate was 12% (5/41) after improvement by the procedure. Minor complications (one case of bleeding, three cases of pneumoperitoneum, and one case of peritonitis) occurred after the procedure in five patients (11%), but there were no major complications. Conclusions: Single-step EUS-guided transmural drainage can be used to treat pancreatic pseudocysts with acceptable feasibility, efficacy, and safety. (Gut Liver 2010;4:524-529)

Background EUS-guided biliary drainage (EUS-BD) with the use of a fully covered metal stent can result in a large-diameter fistula between the bile duct and the duodenum or stomach. This sustainable fistula may constitute a new endoscopic route to the bile duct. Objective To assess the feasibility of performing endoscopic procedures through EUS-guided choledochoduodenostomy or hepaticogastrostomy with a fully covered self-expandable metal stent (FCSEMS). Design Observational pilot study. Setting Tertiary-care referral center. Patients This study involved 3 consecutive patients who underwent EUS-BD with an FCSEMS for biliary decompression. Interventions One to four weeks after EUS-BD with an FCSEMS, endoscopic procedures for the bile duct were performed through the sinus tract to evaluate and manage intrabiliary lesions. Main Outcome Measurements Technical success and procedural complications of endoscopic procedures through EUS-BD with an FCSEMS. Results Endoscopic procedures were completed through the sinus tract of EUS-BD. Two patients underwent photodynamic therapy for tumor bleeding in the common bile duct and argon plasma coagulation on the biliary intraductal papillary mucinous neoplasm repeatedly under direct visual guidance. In the other patient, photodynamic therapy was performed on the malignant hilar stricture through EUS-guided hepaticogastrostomy with an FCSEMS. Limitations Small sample size, pilot study. Conclusions EUS-BD with an FCSEMS may result in a large-diameter sustainable fistula. Endoscopic intervention through this fistula seems to be feasible and useful for the management of intrabiliary lesions.

Background Fully covered self-expandable metal stent (FCSEMS) placement has recently been tried in the management of refractory pancreatic-duct strictures associated with advanced chronic pancreatitis. The major limitation of FCSEMSs was frequent migration. Objective To assess the safety, migration rate, and removability of modified FCSEMSs with antimigration features used for the treatment of benign pancreatic-duct strictures. Design Prospective study. Setting Tertiary academic center. Patients Thirty-two patients with chronic painful pancreatitis and dominant ductal stricture. Interventions Transpapillary endoscopic placement of FCSEMSs in the pancreatic duct with removal after 3 months. Main Outcome Measurements Technical and functional success and adverse events associated with the placement of metal stents. Results FCSEMSs were successfully placed in all patients through the major (n = 27) or minor (n = 5) duodenal papilla. All patients achieved pain relief from stent placement. There was no occurrence of stent-induced pancreatitis or pancreatic sepsis. No stent migrated, and all stents were easily removed. Follow-up ERCP 3 months after stent placement showed resolution of duct strictures in all patients. Pancreatograms obtained at FCSEMS removal displayed de novo focal pancreatic duct strictures in 5 patients, but all were asymptomatic. Limitations No long-term follow-up. Conclusions Temporary 3-month placement of FCSEMSs was effective in resolving pancreatic-duct strictures in chronic pancreatitis, with an acceptable morbidity profile. Modified FCSEMSs can prevent stent migration, but may be associated with de novo duct strictures. Further trials are needed to assess long-term safety and efficacy.