IgG4‐related sclerosing cholangitis and autoimmune pancreatitis: Histological assessment of biopsies from Vater's ampulla and the bile duct

Kawakami, Hiroshi; Zen, Yoh; Eto, Kazunori; Haba, Shin; Yamato, Hiroaki; Shinada, Keisuke; Kubota, Kanako; Asaka, Masahiro

doi:10.1111/j.1440-1746.2010.06346.x

Cited by 113 publications

(71 citation statements)

References 26 publications

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“…In this clinical setting, bile duct biopsy and biliary cytology are more useful than liver needle biopsy (Fig. 4a, b) [68]. Although some patients may require steroid trials, if imaging abnormalities do not improve in the first 2-3 weeks after the initiation of steroid therapy, the diagnosis of ISC will be questioned.…”

Section: Differential Diagnosis Cholangiocarcinomamentioning

confidence: 99%

IgG4-related sclerosing cholangitis: all we need to know

2016

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Our knowledge and experience of IgG4-related sclerosing cholangitis (ISC) have expanded in the last decade. ISC is one of the common organ manifestations of IgG4-related disease (IgG4-RD); approximately 60 % of patients with this systemic condition have ISC in the proximal and/or distal bile ducts. ISC needs to be discriminated from primary sclerosing cholangitis, cholangiocarcinoma, and other rare forms of lymphoplasmacytic cholangiopathy (e.g., follicular cholangitis and sclerosing cholangitis with granulocytic epithelial lesions). Its diagnosis requires a multidisciplinary approach, in which serology, histology, and imaging play crucial roles. Treatments with high-dose corticosteroids typically lead to the rapid and consistent induction of disease remission. Another promising therapeutic approach is B-cell depletion with rituximab. Although disease relapse is relatively common, provided that appropriate treatments are administered, ISC is considered a ''benign'' disease with a low risk of liver failure and biliary malignancy. Its molecular pathology is characterized by Th2-dominant immune reactions, regulatory T-cell activation, and CCL1-CCR8 interactions. Particular subsets of B cells such as plasmablasts and regulatory B cells also expand. A recent global proteomic study demonstrated that three significantly activated immunological cascades in ISC were all B-cell-or immunoglobulin-related (Fc-gamma receptormediated phagocytosis, B-cell receptor signaling pathway, and Fc-epsilon receptor I signaling pathway), suggesting the crucial roles of B cells in the underlying immune reactions. Despite the expansion of our knowledge of the pathophysiology of ISC, the exact role of IgG4 remains unclear. A better understanding of its immunopathology will offer some potential drug targets for this emerging biliary disease.

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Section: Differential Diagnosis Cholangiocarcinomamentioning

confidence: 99%

IgG4-related sclerosing cholangitis: all we need to know

2016

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“…6,8,10,12,[15][16][17]27,28,30,32,34,35,49,51,52,[55][56][57][58][59][60][61][62][63] Consensus statement on the pathology of IgG4-related disease V Deshpande et al which is designed to bind to the Fc portion of IgG4 molecules. 7,10,11,31,40,41 Polyclonal antibodies have been also used by some groups.…”

Section: Technical Issues-igg4 Immunostainmentioning

confidence: 99%

Consensus statement on the pathology of IgG4-related disease

et al. 2012

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IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 þ plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.

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“…6 Cholangiography and IDUS findings with biopsy have been useful to distinguish IgG4-SC from cholangiocarcinoma and PSC. [7][8][9][10][11] A bile duct wall thicker than 0.8 mm in regions of non-stricture on a cholangiogram and IDUS is suggestive of IgG4-SC. 8 Bile duct biopsies are useful for ruling out malignant disease in some cases.…”

Section: Discussionmentioning

confidence: 99%

Usefulness of Steroid Administration for Diagnosis of IgG4-Related Sclerosing Cholangitis

Kubo

Suzuki

Kobayashi

et al. 2012

International Surgery

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Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is one of the IgG4-related systemic sclerosing diseases and responds well to steroid therapy. A 58-year-old male was admitted with hilar bile duct stenosis revealed by computed tomography. We performed percutaneous transhepatic right portal vein embolization (PTPE) and scheduled a right hepatectomy because a hilar cholangiocarcinoma was first suspected. However, there was no cytologic evidence of malignancy and serum IgG4 was elevated. Steroid therapy was initiated after PTPE. There was no evidence of bile duct stenosis after 4 weeks. Improving diagnostic technique, IgG4-SC was diagnosed and treated with steroid therapy. In some cases, we couldn't deny the malignancy and performed unnecessary resection. We recommend that steroid administration while waiting for the liver volume to increase after PTPE is useful. The therapy aids in the diagnosis of bile duct stenosis, which has value for a hilar bile duct limit type of IgG4-SC, as in the case reported here.

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IgG4‐related sclerosing cholangitis and autoimmune pancreatitis: Histological assessment of biopsies from Vater's ampulla and the bile duct

Abstract: Background and Aim. Autoimmune pancreatitis is commonly associated with immunoglobulin

Cited by 113 publications

References 26 publications

IgG4-related sclerosing cholangitis: all we need to know

IgG4-related sclerosing cholangitis: all we need to know

Consensus statement on the pathology of IgG4-related disease

Usefulness of Steroid Administration for Diagnosis of IgG4-Related Sclerosing Cholangitis

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