Management, outcomes and survival of an Australian IgG4-SC cohort: The MOSAIC study.

BACKGROUND AND AIMS: IgG4 sclerosing cholangitis (IgG4-SC) is the biliary component of the multisystem IgG4-related disease. We aimed to investigate the clinical features, demographics, treatment response and outcomes of IgG4-SC in a large Australian cohort. METHODS: We conducted nationwide retrospective cohort via the Australian Liver Association Clinical Trials Network (ALA-CRN). 39 sites were invited to participate. IgG4-SC was defined by the clinical diagnostic criteria established by the Japanese Biliary Association in 2012. Data were collected on patient demographic, clinical and laboratory information, presenting features, response to therapy and clinical outcomes. RESULTS: 67 patients meet inclusion criteria from 22 sites. 76% were male with mean age of 63.3 ± 14.5 years and a median IgG4 level of 3.6 g/L [0.09-67.1]. The most frequent presenting symptom was jaundice (62%) and abdominal pain (42%) and Type 1 biliary stricturing (52%) at the distal common bile duct was the most frequent biliary tract finding. Prednisolone was used as a primary treatment in 61 (91%) and partial or complete response occurred in 95% of subjects. Relapse was common (42%) in those who ceased medical therapy. After a median follow up of 3.9 years there was one hepatocellular carcinoma and no cholangiocarcinomas. CONCLUSIONS: Our study confirms the preponderance of IgG4-SC in males and highlights the steroid response nature of this condition although relapse is common after steroid cessation. Progression to malignancy was uncommon.

Outcomes of preoperative biliary drainage from a single tertiary center: Is there still a role for plastic stents?

OBJECTIVES: Preoperative biliary drainage (PBD) can relieve symptoms of cholestasis, but carries risk of procedural complications. Metal stents have wider lumens and longer patency, although plastic stents (PS) remain in use. We reviewed the outcomes after PBD in patients with cholestasis. METHODS: Patients with symptomatic cholestasis who were likely to wait for over 2 weeks before surgery and were thus treated with PBD between January 2011 and May 2015 were included. Patients were evaluated for stenting-related complications, time interval to surgery, resection rate, improvement in bilirubin level and surgical complications. RESULTS: Forty patients underwent PBD by endoscopic retrograde cholangiopancreatography (ERCP). Of these, 36 patients received the placement of PS, one received a metal stent and the remaining three required percutaneous drainage due to unsuccessful biliary cannulation. Serum bilirubin declined from 172 µmol/L (baseline) to 14 µmol/L at 30 days (P < 0.0001). Median time interval from ERCP to surgery in all patients was 5 weeks (range 2-36 weeks). Preoperative stenting-related complications occurred in seven patients after a median of 3 weeks (range 1-6 weeks). Median time to surgery was similar in patients with and without stenting-related complications (5 weeks vs 4 weeks, respectively, P = 0.33). Surgery was completed in 32 (80%) patients, with a post-Whipple complication rate of 53%. CONCLUSIONS: PBD using mostly PS was effective in reducing bilirubin levels and did not detrimentally affect time interval to surgery. Median time interval to stenting-related complication occurred after 3 weeks, suggesting PS may be most useful for short-term PBD.

Identification and staging of pancreatic tumours using computed tomography, endoscopic ultrasound and mangafodipir trisodium-enhanced magnetic resonance imaging.

Pancreatic malignancy can be staged by a number of different investigations, either alone or in combination. The purpose of the present study was to compare the use of endoscopic ultrasound, CT and mangafodipir trisodium-enhanced MRI for the staging of pancreatic malignancy, particularly with respect to determining resectability prior to surgery. Twenty-seven patients referred for the investigation of a suspected pancreatic malignancy were entered into the trial. All patients had contrast-enhanced CT, gadolinium and mangafodipir trisodium-enhanced MRI, and endoscopic ultrasound (EUS). Images were assessed for nodal staging, tumour staging and resectability for each investigation, and the results compared with findings at surgery. The results for the accuracy of MRI, CT and EUS, in detecting T4 disease versus T3 or lower was 78, 79 and 68%, respectively; nodal involvement was 56, 63 and 69%, respectively; and overall resectability (including the T stage, presence of involved nodes and metastases) was 83, 76 and 63%, respectively. There was no significant difference demonstrated between the three tests. The present study suggests that for patients referred for investigation and staging of pancreatic malignancy, EUS and MRI scanning convey little advantage over contrast-enhanced CT. Furthermore, although mangafodipir trisodium improved the conspicuity of pancreatic tumours, it has little influence on T staging.