Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

Okazaki, Kazuichi; Yanagawa, Masahito; Mitsuyama, Toshiyuki; Uchida, Kazushige

doi:10.5009/gnl14107

Cited by 34 publications

(23 citation statements)

References 64 publications

(111 reference statements)

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“…Typical examples are: The IgG4‐related disease that affects, in the same patient, the biliary tract, such as IgG4‐related sclerosing cholangitis, and the pancreas, such as lympho‐plasmatic sclerosing autoimmune pancreatitis (Okazaki et al. ). Pure mucin‐secreting cholangiocarcinoma (CCA) showing extensive similarities with ductal pancreatic adenocarcinoma. Preneoplastic lesions of bile ducts and pancreatic ducts (Nakanuma et al. ). …”

Section: Discussionmentioning

confidence: 99%

Progenitor cell niches in the human pancreatic duct system and associated pancreatic duct glands: an anatomical and immunophenotyping study

et al. 2015

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Pancreatic duct glands (PDGs) are tubule-alveolar glands associated with the pancreatic duct system and can be considered the anatomical counterpart of peribiliary glands (PBGs) found within the biliary tree. Recently, we demonstrated that endodermal precursor niches exist fetally and postnatally and are composed functionally of stem cells and progenitors within PBGs and of committed progenitors within PDGs. Here we have characterized more extensively the anatomy of human PDGs as novel niches containing cells with multiple phenotypes of committed progenitors. Human pancreata (n = 15) were obtained from cadaveric adult donors. Specimens were processed for histology, immunohistochemistry and immunofluorescence. PDGs were found in the walls of larger pancreatic ducts (diameters > 300 μm) and constituted nearly 4% of the duct wall area. All of the cells identified were negative for nuclear expression of Oct4, a pluripotency gene, and so are presumably committed progenitors and not stem cells. In the main pancreatic duct and in large interlobular ducts, Sox9(+) cells represented 5-30% of the cells within PDGs and were located primarily at the bottom of PDGs, whereas rare and scattered Sox9(+) cells were present within the surface epithelium. The expression of PCNA, a marker of cell proliferation, paralleled the distribution of Sox9 expression. Sox9(+) PDG cells proved to be Pdx1(+) /Ngn3(+/-) /Oct4A(-) . Nearly 10% of PDG cells were positive for insulin or glucagon. Intercalated ducts contained Sox9(+) /Pdx1(+) /Ngn3(+) cells, a phenotype that is presumptive of committed endocrine progenitors. Some intercalated ducts appeared in continuity with clusters of insulin-positive cells organized in small pancreatic islet-like structures. In summary, PDGs represent niches of a population of Sox9(+) cells exhibiting a pattern of phenotypic traits implicating a radial axis of maturation from the bottoms of the PDGs to the surface of pancreatic ducts. Our results complete the anatomical background that links biliary and pancreatic tracts and could have important implications for the common patho-physiology of biliary tract and pancreas.

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Section: Discussionmentioning

confidence: 99%

Progenitor cell niches in the human pancreatic duct system and associated pancreatic duct glands: an anatomical and immunophenotyping study

et al. 2015

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“…Aside from the pancreas, the bile ducts are the most commonly involved organ in IgG4-related disease; about 60%-80% of patients with type 1 autoimmune pancreatitis have involvement of the hepatobiliary tract. However, IgG4-related sclerosing cholangitis can also occur without pancreatic involvement, making accurate diagnosis in these patients particularly difficult (47,48). Both the intrahepatic and extrahepatic segments can be affected, producing dense bile duct infiltration by IgG4-positive plasma cells and extensive fibrosis.…”

Section: Igg4-related Sclerosing Cholangitismentioning

confidence: 99%

IgG4-related Disease from Head to Toe

Martínez-de-Alegría¹,

et al. 2015

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“…Misdiagnosed type 1 pancreatitis leads to surgery for suspected pancreatic adenocarcinoma in many cases, as described in the literature [1]. Hopefully, this situation is much less common for IgG4-mediated biliary tract involvement.…”

Section: Discussionmentioning

confidence: 99%

“…The salivary glands, retroperitoneum, retroocular tissue, pancreas and hepatobiliary system can all be affected [1, 2]. Type 1 autoimmune pancreatitis is the most studied disease but is still rare [3].…”

Section: Introductionmentioning

confidence: 99%

Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma

Bochatay¹,

Majno²,

Giostra³

et al. 2016

Case Rep Gastroenterol

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IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria. The patient presented with progressive cholestasis identified by MR cholangiography as an isolated hilar mass responsible for dilatation of the left and right intrahepatic bile duct suspicious for a Klatskin tumor. The IgG4 blood level was normal as was biliary cytology. The patient underwent right portal embolization followed by right extended hepatectomy. Pathologic examination found no tumor but intense fibrosclerotic infiltration with a marked inflammatory infiltrate characterized by IgG4-positive plasmocytes. Despite immunosuppressive treatment, cholestasis was never controlled and successive biopsies of the remaining liver showed progressive cholestasis, liver infiltrate and no bile duct regeneration. The patient finally presented an upper gastrointestinal hemorrhage leading to death 4 months after hepatectomy and appropriate immunosuppressive therapy.

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Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

Cited by 34 publications

References 64 publications

Progenitor cell niches in the human pancreatic duct system and associated pancreatic duct glands: an anatomical and immunophenotyping study

Progenitor cell niches in the human pancreatic duct system and associated pancreatic duct glands: an anatomical and immunophenotyping study

IgG4-related Disease from Head to Toe

Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma

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