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Type 1 Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis Is Associated With Extrapancreatic Organ Failure, Malignancy, and Mortality in a Prospective UK Cohort

Huggett, Matthew T BSc, BM, PhD, MRCP1, 2, 8; Culver, E L BSc, MBChB, MRCP3, 4, 8; Kumar, M MBBS, MRCP2; Hurst, J M PhD5; Rodriguez-Justo, M MBChB, MSc, FRCPath6; Chapman, M H PhD, MRCP2; Johnson, G J MSc, MD, FRCP2; Pereira, S P PhD, FRCP, FRCPE1, 2; Chapman, R W MD, FRCP3, 4; Webster, George J M BSc, MD, FRCP2, 9; Barnes, E BSc, MBBS, PhD, MRCP3, 4, 7, 9

American Journal of Gastroenterology: October 2014 - Volume 109 - Issue 10 - p 1675–1683
doi: 10.1038/ajg.2014.223
Pancreas and Biliary Tract

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.

1 UCL Institute for Liver and Digestive Health, University College London, London, UK

2 Department of Gastroenterology and Hepatology, University College Hospital, London, UK

3 Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK

4 NDM Oxford University, Oxford Martin School, Oxford University, Oxford, UK

5 Institute of Emerging Diseases, Oxford Martin School, Oxford University, Oxford, UK

6 Department of Pathology, University College Hospital, London, UK

7 Oxford NIHR BRC, Oxford University, Oxford, UK

8 M.T. Huggett and E.L. Culver contributed equally to this work

9 G.J.M. Webster and E. Barnes contributed equally to this work

Correspondence: George J.M. Webster, BSc MD FRCP, Department of Gastroenterology and Hepatology, University College Hospital, Ground Floor West, 250 Euston Road, London NW1 2PG, UK. E-mail:

Received 24 February 2014; accepted 25 July 2014

published online 26 August 2014

© The American College of Gastroenterology 2014. All Rights Reserved.
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