Immunoglobulin G4 associated cholangitis: Description of an emerging clinical entity based on review of the literature

Bjõrnsson, Einar; Chari, Suresh T.; Smyrk, Thomas C.; Lindor, Keith D.

doi:10.1002/hep.21685

Cited by 224 publications

(161 citation statements)

References 56 publications

(200 reference statements)

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“…Björnsson et al argued that lower bile duct stricture should be a part of AIP and not included with IgG4-SC. 19 The present study, however, showed that inflammatory wall thickening of the lower bile duct possibly influences lower bile duct stricture based on IDUS findings and abundant IgG4-bearing plasma cell infiltration in trans-papillary lower bile duct biopsies. Nakazawa et al considered biliary stricture limited to the intra-pancreatic area as IgG4-SC.…”

Section: Discussioncontrasting

confidence: 69%

Mechanisms of Lower Bile Duct Stricture in Autoimmune Pancreatitis

Watanabe

Maruyama²,

Ito³

et al. 2014

Pancreas

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ObjectivesWe attempted to clarify the mechanism underlying lower bile duct stricture in autoimmune pancreatitis. MethodsImaging and histology of the bile duct were assessed for 73 patients with autoimmune pancreatitis to clarify whether IgG4-related biliary inflammation or pancreatic head swelling is associated with lower bile duct stricture. ResultsLower bile duct stricture was found in 59 patients (81%). Pancreatic head swelling was significantly more frequent among patients with lower bile duct stricture than those without (53 (90%) versus 4 (29%); p<0.01). Intraductal ultrasonography revealed lower bile duct wall thickening in 21 of 22 patients with lower bile duct stricture (95%), and lower bile duct wall of patients with pancreatic head swelling was significantly thicker than those without (p=0.028). Among 38 patients with lower bile duct biopsies, 14 (37%) exhibited abundant IgG4-bearing plasma cell infiltration. Among lower bile duct 4 stricture patients, an IgG4-related inflammation seemed to exert a dominant effect under limited conditions, including concomitant middle bile duct stricture and neither pancreatic swelling nor pancreatic duct stricture in the head region. ConclusionsBoth pancreatic head swelling and IgG4-related biliary inflammation influence the occurrence of lower bile duct stricture, which may be included in IgG4-related sclerosing cholangitis. Pancreatic head swelling affects IgG4-related biliary wall thickening.

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Section: Discussioncontrasting

confidence: 69%

Mechanisms of Lower Bile Duct Stricture in Autoimmune Pancreatitis

Watanabe

Maruyama²,

Ito³

et al. 2014

Pancreas

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“…Patients with ISC typically present with obstructive jaundice [14,15,27]. This presentation is particularly common in patients with concomitant pancreatitis.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Serum IgG4 elevations are the most sensitive and specific non-invasive examination for the diagnosis of ISC [14,15,27]. By using a typical cut-off value of 135 or 140 mg/dl, a previous study demonstrated that approximately 80 % of patients with ISC had elevated levels of IgG4 [28].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Subsequent pathological studies identified similar IgG4-related fibroinflammatory conditions in various organs outside the pancreas [2][3][4][5][6][7][8][9][10], eventually leading to the recognition of a systemic condition named IgG4-related disease (IgG4-RD) [11,12]. During this process, the bile duct appeared to be one of the target organs commonly affected by this condition [13][14][15]. Sclerosing cholangitis is a central biliary manifestation of IgG4-RD [13][14][15].…”

Section: Introductionmentioning

confidence: 99%

“…During this process, the bile duct appeared to be one of the target organs commonly affected by this condition [13][14][15]. Sclerosing cholangitis is a central biliary manifestation of IgG4-RD [13][14][15]. Although our clinical experience and knowledge of IgG4-related sclerosing cholangitis (ISC) have expanded rapidly in the last decade, many questions remain to be answered.…”

Section: Introductionmentioning

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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