<scp>IgG</scp>4‐related sclerosing cholangitis without obvious pancreatic lesion: Difficulty in differential diagnosis

Matsubayashi, Hiroyuki; Uesaka, Katsuhiko; Sugiura, Teichi; Ohgi, Kunio; Sasaki, Keiko; Ono, Hiroyuki

doi:10.1111/1751-2980.12149

Cited by 16 publications

(12 citation statements)

References 25 publications

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“…It has been reported that AIP is absent in 10% of IAC cases [23,24]. A recent report described a series of six Japanese cases of IAC without clinically manifest AIP [25] and the current case is similar to the cases in this report.…”

Section: Discussionsupporting

confidence: 87%

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

Elsabaawy¹,

Abdelsameea²,

Abdallah³

et al. 2017

J Mol Biomark Diagn

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Section: Discussionsupporting

confidence: 87%

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

Elsabaawy¹,

Abdelsameea²,

Abdallah³

et al. 2017

J Mol Biomark Diagn

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“…Common examples are round or wedge-shaped renal cortical lesions, peripheral cortical nodules, mass-like lesions, and pelvic wall thickening in the kidneys; soft-tissue masses surrounding the aorta and its branches in the retroperitoneum and mesentery; and lymphadenopathy [41][42][43][44]. An imaging diagnosis of ISC becomes highly challenging in patients with no recognizable lesion in other organs [45,46]. The image findings useful for the diagnosis of ISC include multifocal biliary strictures, a markedly thickened bile duct wall (mean wall thickness, 4.9 mm), a smooth outer margin, a narrow but visible lumen, hyperenhancement during the late arterial phase, homogeneous hyperenhancement during the delayed phase, concurrent gallbladder wall thickening, and no vascular invasion (Fig.…”

Section: Ct/mrmentioning

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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“…The present case showed stenoses of the bile duct but no elevated serum IgG4 concentrations or abnormal imaging findings of the pancreas. Similarly, some previous IgG4-SC cases were also reported to show no elevated serum IgG4 concentrations [6, 7] or no abnormal imaging findings of the pancreas [8, 9]; it is therefore necessary to diagnose IgG4-SC carefully. Nakazawa et al [10] classified IgG4-SC into 4 types when making a differential diagnosis based on the cholangiographic features: stricture located in the lower part of the CBD is type 1, stricture that is diffusely distributed in the intra- and extrahepatic bile ducts is type 2, stricture distributed in both the hilar hepatic region and the lower part of the CBD is type 3, and stricture of the bile duct in the hilar hepatic region is type 4.…”

Section: Discussionmentioning

confidence: 99%

Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis

Suzumura

Hatano

Tada

et al. 2018

Case Rep Gastroenterol

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A 75-year-old male was admitted to our hospital because of bile duct stenosis. He had no medical history of autoimmune disease. The level of tumor markers, serum IgG, and IgG4 were within normal ranges. Computed tomography showed perihilar and distal bile duct stenosis and wall thickening without swelling or abnormal enhancement of the pancreas. Endoscopic retrograde cholangiopancreatography showed perihilar and distal bile duct stenosis. A biopsy and cytology from the distal bile duct stenosis suggested adenocarcinoma, and cytology from the perihilar bile duct also suggested adenocarcinoma. A preoperative diagnosis of perihilar and distal bile duct cancer was made, and the patient underwent left hepatectomy and pancreaticoduodenectomy. Resected specimens showed wall thickening in the perihilar and distal bile duct; however, tumors were unclear. A histopathological examination revealed lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis in the perihilar and distal bile ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the perihilar and distal bile ducts. Lymphoplasmacytic infiltration, inflammatory change, storiform fibrosis, and obliterative phlebitis were shown in the pancreas. A final diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC) with autoimmune pancreatitis was made. We herein report a case in which a preoperative diagnosis of IgG4-SC was difficult due to normal serum IgG4 levels and no obvious pancreatic lesion.

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IgG4‐related sclerosing cholangitis without obvious pancreatic lesion: Difficulty in differential diagnosis

Cited by 16 publications

References 25 publications

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

IgG4-related sclerosing cholangitis: all we need to know

Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis

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