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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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.