Igg4 Related Disease – A Retrospective Descriptive Study Highlighting Canadian Experiences In Diagnosis And Management

Conflicts of Interest Ethics: Silencing Expertise in the Development of International Clinical Practice Guidelines

Of those patients in our cohort who did not meet classic criteria for AIP, 3 had sclerosing cholangitis with high IgG4. Their response to immunosuppression was not dramatic, and notably they had limited radiologic evidence of more classic AIP. This cautions clinicians about the initial optimism for steroids in a subset of PSC patients with elevated IgG4, and again reiterates the need for prospective research in this area. Additionally, it favours the importance of radiologic features of autoimmune pancreatitis in predicting outcome from steroid treatment. Retrospective descriptive review is inevitably limited as compared to prospective studies, but for a rare disease managed by many clinicians, across in-patient and out-patient settings, it remains distinctly hard to be prospective in ones approach. Nevertheless clinicians and patients are in need of descriptions of disease that match their own experience and practice, as is the case here: our data therefore adds to the breadth of experience described for this difficult and often enigmatic disease, and highlights how patients have presented and been managed over time, as concepts about the disease have evolved, and clinicians have grown in their experience. Ideally specialist clinics would triage referrals and facilitate more prospective studies to address more closely the role of radiology over histology in diagnosing this syndrome. Clearly our approach cant formally validate the scoring systems in use (and isnt designed to; nor is it designed to utilise every scoring system presently available not least because not all clinical information was available to us, and this obstacle could not be overcome retrospectively), but nevertheless we can demonstrate their applied utility, which reflects the interest of clinicians. Similarly our observations largely reflect the type 1 AIP disease spectrum, and there will inevitably be patients presenting with type 2 disease (albeit much less common) who do not adequately fit into our description. Autoimmune pancreatitis is notably a highly steroid sensitive disease. In our series there was a lower than expected steroid response to treatment. The likely explanation for this, once again, reflects the real world practice described. In settings where patients present to a variety of clinicians, in which referral pathways are set and potentially limited by pressure on resources, it is more likely that patients will have a delay in diagnosis and hence a potential impact on treatment. Additionally without clinical confidence from treating prior patients, clinicians may not always capture diagnosis accurately i.e. there is a risk of over-diagnosis. Our series, and many others, are therefore of importance in repeatedly highlighting this disease once again to a general audience.

a cool way to improve http://www.biomedcentral.com/1471-230X/13/168

Critical revision of the article for important intellectual content: D.J. Jones, A.N. Barkun, Y. Lu, R. Enns, P. Sinclair, I. Gralnek, M. Bardou, E.J. Kuipers. Final approval of the article: D.J. Jones, A.N. Barkun, Y. Lu, R. Enns, P. Sinclair, I. Gralnek, M.

funny post http://annals.org/article.aspx?articleid=1170881


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