Follicular cholangitis and pancreatitis – clinicopathological features and differential diagnosis of an under‐recognized entity

Zen, Yoh; Ishikawa, Akira; Ogiso, Seiji; Heaton, Nigel; Portmann, Bernard

doi:10.1111/j.1365-2559.2011.04078.x

Cited by 58 publications

(67 citation statements)

References 27 publications

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“…The inflammatory infiltrate in follicular cholangitis is more lymphocytic and less plasmacytic than that in ISC. A similar inflammatory process rich in lymphoid follicles may develop in the pancreas and gallbladder, referred to as follicular pancreatitis and follicular cholecystitis, respectively [73,77]. Whether or not serum IgG4 levels can be elevated in this condition is unknown.…”

Section: Follicular Cholangitismentioning

confidence: 99%

“…A tissue examination is essential for a conclusive diagnosis. Follicular cholangitis usually affects perihilar large bile ducts, but broad cholangiopathy mimicking ISC or PSC is rarely encountered [73]. A characteristic histological feature is duct-centered inflammation associated with many lymphoid follicles (Fig.…”

Section: Follicular Cholangitismentioning

confidence: 99%

“…This condition is estimated to be rare with less than a dozen cases being reported [73][74][75][76]. Patients typically lack any serological autoimmune abnormalities or associated immune-mediated conditions in other organs.…”

Section: Follicular Cholangitismentioning

confidence: 99%

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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: Follicular Cholangitismentioning

confidence: 99%

Section: Follicular Cholangitismentioning

confidence: 99%

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IgG4-related sclerosing cholangitis: all we need to know

2016

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“…Immunohistochemically, the infiltrating lymphocytes consist of CD3-positive T cells and CD20-positive B cells; CD4, CD8, and CD79a are also expressed. The distribution of λ-chain and κ-chain cells supports the polyclonal nature of B cells and plasma cells [103]. Bcl-2 is not expressed in the germinal center [103,105], and IgG4-positive plasma …”

Section: Follicular Cholangitismentioning

confidence: 96%

“…3). This extremely rare clinicopathologic entity preferentially affects the hilar bile ducts of adults, represents radiological features suggestive of hilar cholangiocarcinoma, and may progress to cirrhosis if left untreated [103]. Since the first case Lymphoid follicles have also been observed in the gallbladder mucosa in some patients with follicular cholangitis [103].…”

Section: Follicular Cholangitismentioning

confidence: 99%

Adult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma

Canh

Harada

2016

Med Mol Morphol

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Biliary epithelial cells preferentially respond to various insults under chronic pathological conditions leading to reactively atypical changes, hyperplasia, or the development of biliary neoplasms (such as biliary intraepithelial neoplasia, intraductal papillary neoplasm of the bile duct, and cholangiocarcinoma). Moreover, benign biliary strictures can be caused by a variety of disorders (such as IgG4-related sclerosing cholangitis, eosinophilic cholangitis, and follicular cholangitis) and often mimic malignancies, despite their benign nature. In addition, primary sclerosing cholangitis is a well-characterized precursor lesion of cholangiocarcinoma and many other chronic inflammatory disorders increase the risk of malignancies. Because of these factors and the changes in biliary epithelial cells, biliary strictures frequently pose a diagnostic challenge.Although the ability to differentiate neoplastic from non-neoplastic biliary strictures has markedly progressed with the advance in radiological modalities, brush cytology and bile duct biopsy examination remains effective. However, no single modality is adequate to diagnose benign biliary strictures because of the low sensitivity. Therefore, understanding the underlying causes by compiling the entire clinical, laboratory, and imaging data; considering the underrecognized causes; and collaborating between experts in various fields including cytopathologists with multiple approaches is necessary to achieve an accurate diagnosis.

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Histology of Autoimmune Pancreatitis

Gupta

2018

The Pancreas

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Follicular cholangitis and pancreatitis – clinicopathological features and differential diagnosis of an under‐recognized entity

Abstract: This study suggests that a disease entity which can be named follicular cholangitis and pancreatitis exists and may be under-recognized. The disease mainly affects the hilar bile ducts and pancreatic head in adults.

Cited by 58 publications

References 27 publications

IgG4-related sclerosing cholangitis: all we need to know

IgG4-related sclerosing cholangitis: all we need to know

Adult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma

Histology of Autoimmune Pancreatitis

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