scholarly journals IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy

2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Malene Theilmann Thinesen ◽  
Ove B. Schaffalitzky de Muckadell ◽  
Sönke Detlefsen
Keyword(s):  
Bile Duct ◽  
Liver Biopsy ◽  
Hot Spots ◽  
Sclerosing Cholangitis ◽  
Bile Ducts ◽  
Bile Duct Carcinoma ◽  
Duct Carcinoma ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs. When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis. A 74-year-old male complained of dysphagia and abdominal pain. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis. Liver biopsy showed storiform fibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal tract. Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%. Eight months earlier, an inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells. Hot spots revealed 593 IgG4-positive cells and an IgG4/IgG ratio of 92%. The serum IgG4 of the patient was elevated nearly 10 times upper limit of normal. The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was good response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease.

scholarly journals Mucin-producing Bile Duct Carcinoma Arising from Primary Sclerosing Cholangitis: A Case Report

2007 ◽  
Vol 74 (1) ◽  
pp. 61-64 ◽  
Author(s):  
Shigeki Yokomuro ◽  
Yasuo Arima ◽  
Yoshiaki Mizuguchi ◽  
Tetsuya Shimizu ◽  
Yutaka Kawahigashi ◽  
...  
Keyword(s):  
Case Report ◽  
Bile Duct ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Bile Duct Carcinoma ◽  
Duct Carcinoma

Simultaneous occurrence of autoimmune pancreatitis and sclerosing cholangitis as immune-related adverse events of pembrolizumab

2021 ◽  
Vol 14 (6) ◽  
pp. e243360
Author(s):  
Tsuyoshi Suda ◽  
Masako Kobayashi ◽  
Koji Kurokawa ◽  
Eiki Matsushita
Keyword(s):  
Bile Duct ◽  
Adverse Events ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Intrahepatic Bile Duct ◽  
Simultaneous Occurrence ◽  
Cd8 Cells ◽  
Igg4 Related Disease ◽  
Contrast Enhanced Ct ◽  
Serum Igg4

A 57-year-old man with lung cancer, previously treated with the programmed death-1 inhibitor pembrolizumab, was evaluated for liver injury and acute pancreatitis. Serum IgG4 levels were not elevated. Contrast-enhanced CT showed pancreatic swelling, contrast unevenness in the liver and thickening of the common bile duct and gall bladder. Magnetic resonance cholangial pancreatography revealed beads in the left intrahepatic bile duct and localised narrowing of the head and body of the central pancreatic duct. Endoscopic ultrasound-guided fine-needle and liver needle biopsy showed CD8+ and CD4+ T lymphocyte aggregates, whereas immunostaining revealed greater infiltration by CD8+ cells than CD4+ cells. IgG4-related disease was ruled out based on serum and pathological findings. The patient simultaneously presented with immune-related adverse events, autoimmune pancreatitis-like features and sclerosing cholangitis, which were ameliorated by steroid therapy. CD8+ lymphocytes were the dominant infiltrating cells in autoimmune pancreatitis and sclerosing cholangitis.

Adenocarcinoma with pyloric gland phenotype of the extrahepatic bile ducts: a previously unrecognized and distinctive morphologic variant of extrahepatic bile duct carcinoma

2012 ◽  
Vol 43 (12) ◽  
pp. 2292-2298 ◽  
Author(s):  
Jorge Albores-Saavedra ◽  
Fredy Chablé-Montero ◽  
Nahum Méndez-Sánchez ◽  
Miguel Ángel Mercado ◽  
Mario Vilatoba-Chapa ◽  
...  
Keyword(s):  
Bile Duct ◽  
Bile Ducts ◽  
Extrahepatic Bile Duct ◽  
Bile Duct Carcinoma ◽  
Pyloric Gland ◽  
Duct Carcinoma ◽  
Extrahepatic Bile Duct Carcinoma ◽  
Extrahepatic Bile Ducts

scholarly journals IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma

2021 ◽  
pp. 39-46
Author(s):  
Pham Minh Thong ◽  
Vu Dang Luu ◽  
Thieu-Thi Tra My ◽  
Nguyen Xuan Hien ◽  
Tran Anh Tuan ◽  
...  
Keyword(s):  
Bile Duct ◽  
Sclerosing Cholangitis ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Bile Duct Stenosis ◽  
Diffusion Weighted Images ◽  
Igg4 Related Disease ◽  
Imaging Characteristics ◽  
Serum Igg4 ◽  
Duct Stenosis

IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC.

Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity

2018 ◽  
Vol 17 (5) ◽  
pp. 0-10
Author(s):  
Fernando Martínez-Valle ◽  
Mar Riveiro-Barciela ◽  
María Teresa-Salcedo ◽  
Xavier Merino-Casabiel ◽  
Andreu Fernández-Codina ◽  
...  
Keyword(s):  
Sclerosing Cholangitis ◽  
Good Prognosis ◽  
Biliary Cirrhosis ◽  
Histopathological Findings ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Early Use ◽  
Secondary Biliary Cirrhosis

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance

IgG4-Related Cholangiopathy and Its Mimickers: A Case Report and Review Highlighting the Importance of Early Diagnosis

2017 ◽  
Vol 26 (2) ◽  
pp. 165-173 ◽  
Author(s):  
Kyle Geary ◽  
Cemal Yazici ◽  
Anita Seibold ◽  
Grace Guzman
Keyword(s):  
Early Diagnosis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Treatment Modalities ◽  
Primary Biliary Cholangitis ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy. It is characterized by elevated serum IgG4 levels, an IgG4-positive plasma cell infiltration with storiform fibrosis/obliterative phlebitis of the bile duct wall, and a response to steroids. It is crucial to differentiate IgG4-related cholangiopathy from its mimickers, such as primary biliary cholangitis, secondary biliary cholangitis, primary sclerosing cholangitis, secondary sclerosing cholangitis, and cholangiocarcinoma, because treatment modalities and outcomes of IgG4-related cholangiopathy differ significantly from these disorders. Here, we present an interesting case of IgG4-related cholangiopathy, discuss clinical and pathological features crucial to its early diagnosis, and compare and contrast this condition with its potentially confounding mimickers.

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