OBJECTIVES Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers. METHODS Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1–107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics. RESULTS Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P < 0.01). Malignancy occurred in 11% shortly before or after the diagnosis of IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio = 2.25, CI = 1.12–3.94, P = 0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio = 2.07, CI = 1.07–3.55, P = 0.02). CONCLUSIONS Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up.

CONTEXT Assessment in the workplace is important, but many evaluations have shown that assessor agreement and discrimination are poor. Training discussions suggest that assessors find conventional scales invalid. We evaluate scales constructed to reflect developing clinical sophistication and independence in parallel with conventional scales.METHODS A valid scale should reduce assessor disagreement and increase assessor discrimination. We compare conventional and construct-aligned scales used in parallel to assess approximately 2000 medical trainees by each of three methods of workplace-based assessment (WBA): the mini-clinical evaluation exercise (mini-CEX); the acute care assessment tool (ACAT), and the case-based discussion (CBD). We evaluate how scores reflect assessor disagreement (V j and V j*p ) and assessor discrimination (V p ), and we model reliability using generalisability theory.RESULTS In all three cases the conventional scale gave a performance similar to that in previous evaluations, but the construct-aligned scales substantially reduced assessor disagreement and substantially increased assessor discrimination. Reliability modelling shows that, using the new scales, the number of assessors required to achieve a generalisability coefficient ‡ 0.70 fell from six to three for the mini-CEX, from eight to three for the CBD, from 10 to nine for 'on-take' ACAT, and from 30 to 12 for 'post-take' ACAT. CONCLUSIONSThe results indicate that construct-aligned scales have greater utility, both because they are more reliable and because that reliability provides evidence of greater validity. There is also a wider implication: the disappointing reliability of existing WBA methods may reflect not assessors' differing assessments of performance, but, rather, different interpretations of poorly aligned scales. Scales aligned to the expertise of clinician-assessors and the developing independence of trainees may improve confidence in WBA.assessment

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Background Dominant biliary strictures occur commonly in patients with primary sclerosing cholangitis (PSC), who have a high risk of developing cholangiocarcinoma. The natural history and optimal management of dominant strictures remains unclear, with some reports suggesting that endoscopic interventions improve outcome. Methods We describe a 25 year experience in patients with PSC related dominant strictures at a single tertiary referral centre. Results 128 patients with PSC (64% males, mean age at referral 49 years) were followed for a mean of 9.8 years. 80 patients (62.5%) with dominant biliary strictures had a median of 3 (range 0–34) interventions, compared to 0 (0–7) in the 48 without dominant strictures (p<0.001). Endoscopic interventions included: (i) stenting alone (46%), (ii) dilatation alone (20%), (iii) dilatation and stenting (17%), and (iv) none or failed intervention (17%, of whom most required percutaneous transhepatic drainage). The major complication rate for ERCP was low (1%). The mean survival of those with dominant strictures (13.7 years) was worse than for those without dominant strictures (23 years), with much of the survival difference related to a 26% risk of cholangiocarcinoma developing only in those with dominant strictures. Half of those with cholangiocarcinoma presented within four months of diagnosis of PSC, highlighting the importance of thorough evaluation of new dominant strictures. Conclusions Repeated endoscopic therapy in PSC patients is safe but the prognosis remains worse in the subgroup with dominant strictures. In our series, dominant strictures were associated with a high risk of developing cholangiocarcinoma.

Main RecommendationThere is a need for well-organized comprehensive strategies to achieve good training in ESD. In this context, the European Society of Gastrointestinal Endoscopy (ESGE) have developed a European core curriculum for ESD practice across Europe with the aim of high quality ESD training.Advanced endoscopy diagnostic practice is advised before initiating ESD training. Proficiency in endoscopic mucosal resection (EMR) and adverse event management is recommended before starting ESD trainingESGE discourages the starting of initial ESD training in humans. Practice on animal and/or ex vivo models is useful to gain the basic ESD skills. ESGE recommends performing at least 20 ESD procedures in these models before human practice, with the goal of at least eight en bloc complete resections in the last 10 training cases, with no perforation. ESGE recommends observation of experts performing ESD in tertiary referral centers. Performance of ESD in humans should start on carefully selected lesions, ideally small ( < 30 mm), located in the antrum or in the rectum for the first 20 procedures. Beginning human practice in the colon is not recommended. ESGE recommends that at least the first 10 human ESD procedures should be done under the supervision of an ESD-proficient endoscopist.Endoscopists performing ESD should be able to correctly estimate the probability of performing a curative resection based on the characteristics of the lesion and should know the benefit/risk relationship of ESD when compared with other therapeutic alternatives. Endoscopists performing ESD should know how to interpret the histopathology findings of the ESD specimen, namely the criteria for low risk resection (“curative”), local risk resection, and high risk resection (“non-curative”), as well as their implications. ESD should be performed only in a setting where early and delayed complications can be managed adequately, namely with the possibility of admitting patients to a ward, and access to appropriate emergency surgical teams for the organ being treated with ESD.

Main RecommendationsThe European Society of Gastrointestinal Endoscopy (ESGE) has recognized the need to formalize and enhance training in endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS). This manuscript represents the outcome of a formal Delphi process resulting in an official Position Statement of the ESGE and provides a framework to develop and maintain skills in ERCP and EUS. This curriculum is set out in terms of the prerequisites prior to training; recommended steps of training to a defined syllabus; the quality of training; and how competence should be defined and evidenced before independent practice. 1 Trainees should be competent in gastroscopy prior to commencing training. Formal training courses and the use of simulation in training are recommended. 2 Trainees should keep a contemporaneous logbook of their procedures, including key performance indicators and the degree of independence. Structured formative assessment is encouraged to enhance feedback. There should be a summative assessment process prior to commencing independent practice to ensure there is robust evidence of competence. This evidence should include a review of a trainee’s procedure volume and current performance measures. A period of mentoring is strongly recommended in the early stages of independent practice. 3 Specifically for ERCP, all trainees should be competent up to Schutz level 2 complexity (management of distal biliary strictures and stones > 10 mm), with advanced ERCP requiring a further period of training. Prior to independent practice, ESGE recommends that a trainee can evidence a procedure volume of > 300 cases, a native papilla cannulation rate of ≥ 80 % (90 % after a period of mentored independent practice), complete stones clearance of ≥ 85 %, and successful stenting of distal biliary strictures of ≥ 90 % (90 % and 95 % respectively after a mentored period of independent practice). 4 The progression of EUS training and competence attainment should start from diagnostic EUS and then proceed to basic therapeutic EUS, and finally to advanced therapeutic EUS. Before independent practice, ESGE recommends that a trainee can evidence a procedure volume of > 250 cases (75 fine-needle aspirations/biopsies [FNA/FNBs]), satisfactory visualization of key anatomical landmarks in ≥ 90 % of cases, and an FNA/FNB accuracy rate of ≥ 85 %. ESGE recognizes the often inadequate quality of the evidence and the need for further studies pertaining to training in advanced endoscopy, particularly in relation to therapeutic EUS.

The ability to diagnose AIP based on ERP features alone is limited but can be improved with knowledge of some key features.

Background The endoscopic retrograde cholangiopancreatography (ERCP) direct observation of procedural skills (DOPS) is a 27-item competency assessment tool that was developed to support UK ERCP training. We evaluated validity of ERCP DOPS and competency development during training. Methods This prospective study analyzed ERCP DOPS performed in the UK between July 2016 and October 2018. Reliability was measured using Cronbach’s alpha, and DOPS scores were benchmarked using the contrasting groups method. The percentage of competent scores was averaged for each item, domain, and overall rating, and stratified by lifetime procedure count to evaluate learning curves. Multivariable analyses were performed to identify predictors of DOPS competence. Results 818 DOPS (109 trainees, 80 UK centers) were analyzed. Overall Cronbach’s alpha was 0.961. Attaining competency in 87 % of assessed DOPS items provided the optimal competency benchmark. This was achieved in the domain sequence of: pre-procedure, post-procedure management, endoscopic non-technical skills, cannulation & imaging, and execution of selected therapy, and across all items after 200 – 249 procedures (89 %). After 300 procedures, the benchmark was reached for selective cannulation (89 %), but not for stenting (plastic 73 %; metal 70 %), sphincterotomy (80 %), and sphincteroplasty (56 %). On multivariable analysis, lifetime procedure count (P = 0.002), easier case difficulty (P < 0.001), trainee grade (P = 0.03), and higher lifetime DOPS count (P = 0.01) were predictors of DOPS competence. Conclusion This study provides novel validity, reliability, and learning curve data for ERCP DOPS. Trainees should have a minimum of 300 hands-on ERCP procedures before undertaking summative assessment for independent practice.