IgG4-related disease of the biliary tract and pancreas: clinical and experimental advances

Current Opinion in Gastroenterology
Lowiek M Hubers, U Beuers

Abstract

IgG4-related disease (IgG4-RD) is an immune-mediated disease of unknown cause. It predominantly affects the biliary tract [IgG4-associated cholangitis (IAC)] and pancreas [autoimmune pancreatitis (AIP)] of mostly elderly men. Accurate diagnostic tests are lacking. Patients benefit from predniso(lo)ne treatment. However, disease relapse is often seen. This review will address pathophysiological aspects and advances in diagnostic and therapeutic strategies. The role of IgG1 and IgG4 in the pathophysiology of IgG4-RD was studied in mice which showed more intense organ damage of pancreas and salivary glands when IgG1 rather than IgG4 of patients with IgG4-RD was injected. Coadministration of IgG1+IgG4 led to dampening of IgG1-mediated injury supporting the view that IgG4 exerts immune-dampening effects. IgG4+ B-cell receptor clones identified by next-generation sequencing and the IgG4/IgG RNA ratio in human blood assessed by quantitative PCR were able to accurately distinguish IAC/AIP from primary sclerosing cholangitis or pancreatobiliary malignancies. Long-term treatment with low-dose prednisolone was safe and reduced the number of flare-ups in patients with AIP. Early diagnosis by a novel accurate and easy-to-use qPCR test may p...Continue Reading

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Citations

Sep 4, 2018·Scandinavian Journal of Gastroenterology·Min Liu, Mingju Hao
Jun 22, 2019·Liver International : Official Journal of the International Association for the Study of the Liver·Sina FuchsEdith Hintermann
Jun 20, 2020·United European Gastroenterology Journal·J-Matthias LöhrUNKNOWN UEG guideline working group
Mar 19, 2019·Liver International : Official Journal of the International Association for the Study of the Liver·Timothy KendallGuido Carpino
Mar 9, 2019·The Surgical Clinics of North America·Christina W Lee, Sean Ronnekleiv-Kelly
Jul 25, 2019·Journal of Vascular Surgery Cases and Innovative Techniques·Kuniyasu IkeokaShiro Hoshida

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