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Review
. 2019 Jul;10(3):275-283.
doi: 10.1136/flgastro-2018-101001. Epub 2018 Oct 31.

Diagnosis and management of IgG4-related disease

Vinod S Hegade  1 , Maria B Sheridan  2 , Matthew T Huggett  1
Affiliations
Review

Diagnosis and management of IgG4-related disease

Vinod S Hegade et al. Frontline Gastroenterol. 2019 Jul.

Abstract

IgG subclass 4-related disease (IgG4-RD) is a rare but increasingly recognised fibroinflammatory condition known to affect multiple organs. IgG4-RD is characterised by unique histological features of lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. In this review we describe the pancreaticobiliary manifestations of IgG4-RD, with particular emphasis on type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). AIP and IgG4-SC can pose diagnostic challenges to the clinician as they may mimic pancreatic cancer and primary sclerosing cholangitis, respectively. We discuss current knowledge, clinical diagnostic criteria and recent advances and summarise the evidence base for current therapeutic approaches for AIP and IgG4-SC.

Keywords: pancreas; pancreatic disease; pancreatic fibrosis; pancreatic pathology; pancreatitis.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Radiological features of autoimmune pancreatitis. (A) CT abdomen image showing diffuse enlargement of the pancreas with loss of its lobular contour and presence of a peripancreatic rim/halo (arrow). (B) MR cholangiopancreatography (MRCP) image of the same patient showing distal common bile duct stricture (arrowhead) and narrowing of the main pancreatic duct without upstream dilatation (arrow).
Figure 2
Figure 2
Radiological changes in autoimmune pancreatitis (AIP), presteroid and poststeroid treatment. (A) T1-weighted fat suppressed MRI image showing diffusely low signal intensity (SI) from pancreas (arrow) (normally SI from pancreas is same or higher than SI from liver, marked as*). (B) Four weeks after steroid treatment pancreas volume is decreased (arrow) but SI is returning to normal.
See this image and copyright information in PMC

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