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

2012 ◽  
Vol 97 (2) ◽  
pp. 145-149 ◽  
Author(s):  
Norio Kubo ◽  
Hideki Suzuki ◽  
Tsutomu Kobayashi ◽  
Kenichiro Araki ◽  
Shigeru Sasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Steroid Therapy ◽  
Sclerosing Cholangitis ◽  
Liver Volume ◽  
Immunoglobulin G4 ◽  
Steroid Administration ◽  
Bile Duct Stenosis ◽  
Serum Igg4 ◽  
Hilar Bile Duct ◽  
Duct Stenosis

Abstract 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.

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.

scholarly journals Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis

2018 ◽  
Vol 12 (2) ◽  
pp. 425-431
Author(s):  
Kazuhiro Suzumura ◽  
Etsuro Hatano ◽  
Masaharu Tada ◽  
Hideaki Sueoka ◽  
Hiroshi Nishida ◽  
...  
Keyword(s):  
Bile Duct ◽  
Preoperative Diagnosis ◽  
Sclerosing Cholangitis ◽  
Wall Thickening ◽  
Distal Bile Duct ◽  
Obliterative Phlebitis ◽  
Bile Duct Stenosis ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Duct Stenosis

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.

Differentiation of benign and malignant hilar bile duct stenosis

2016 ◽  
Vol 203 (2) ◽  
pp. 275-282 ◽  
Author(s):  
Xiaolei Liu ◽  
Zhiying Yang ◽  
Haidong Tan ◽  
Chen Shao ◽  
Liguo Liu ◽  
...  
Keyword(s):  
Bile Duct ◽  
Bile Duct Stenosis ◽  
Hilar Bile Duct ◽  
Duct Stenosis

A Case of Xanthogranulomatous Cholecystitis with Hilar Bile Duct Stenosis

2012 ◽  
Vol 45 (11) ◽  
pp. 1083-1090
Author(s):  
Kazuya Nakagawa ◽  
Ryutaro Mori ◽  
Michio Ueda ◽  
Kazunori Nojiri ◽  
Kouichi Taniguchi ◽  
...  
Keyword(s):  
Bile Duct ◽  
Xanthogranulomatous Cholecystitis ◽  
Bile Duct Stenosis ◽  
Hilar Bile Duct ◽  
Duct Stenosis

scholarly journals A CASE OF HILAR BILE DUCT STENOSIS CAUSED BY PERIBILIARY CYSTS

2005 ◽  
Vol 66 (5) ◽  
pp. 1166-1169
Author(s):  
Masatoshi SHIGETA ◽  
Manabu SUDO ◽  
Masahiko ORITA ◽  
Tadahiko ENOKI ◽  
Shinji NOSHIMA ◽  
...  
Keyword(s):  
Bile Duct ◽  
Bile Duct Stenosis ◽  
Hilar Bile Duct ◽  
Peribiliary Cysts ◽  
Duct Stenosis

scholarly journals Role of Peroral Cholangioscopy in the Diagnosis of Primary Sclerosing Cholangitis

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 268
Author(s):  
Toshio Fujisawa ◽  
Mako Ushio ◽  
Sho Takahashi ◽  
Wataru Yamagata ◽  
Yusuke Takasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Chronic Phase ◽  
Active Phase ◽  
Future Studies ◽  
Bile Duct Stenosis ◽  
Peroral Cholangioscopy ◽  
Duct Stenosis

Primary sclerosing cholangitis (PSC) is characterized by idiopathic biliary stricture followed by progressive cholestasis and fibrosis. When diagnosing PSC, its differentiation from other types of sclerosing cholangitis and cholangiocarcinoma is necessary. The cholangioscopic findings of PSC have not been investigated sufficiently. PSC and IgG4-related sclerosing cholangitis are difficult to distinguish by peroral cholangioscopy (POCS), but POCS is useful for excluding cholangiocarcinoma. POCS findings vary according to the condition and stage of disease. In the active phase, findings such as mucosal erythema, ulceration, fibrinous white exudate, and an irregular surface are observed and may reflect strong inflammation in the biliary epithelium. On the other hand, findings such as scarring, pseudodiverticula, and bile duct stenosis appear in the chronic phase and may reflect fibrosis and stenosis resulting from repeated inflammation. Observation of inside the bile duct by POCS might confirm the current PSC activity. Because POCS offers not only information regarding the diagnosis of PSC and PSC-associated cholangiocarcinoma but also the current statuses of biliary inflammation and stenosis, POCS could significantly contribute to the diagnosis and treatment of PSC once the characteristic findings of PSC are confirmed by future studies.

scholarly journals A Case of Chronic Cholecystitis with Peri-hilar Bile Duct Stenosis that was Difficult to Differentiate

2020 ◽  
Vol 96 (1) ◽  
pp. 195-197
Author(s):  
Ichiro Mizushima ◽  
Koichi Kagawa ◽  
Yuki Kasai ◽  
Yukihiro Otani ◽  
Yusaku Tanaka ◽  
...  
Keyword(s):  
Bile Duct ◽  
Chronic Cholecystitis ◽  
Bile Duct Stenosis ◽  
Hilar Bile Duct ◽  
Duct Stenosis

scholarly journals Treatment of Autoimmune Pancreatitis with the Anecdotes of the First Report

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Terumi Kamisawa ◽  
Tadashi Takeuchi
Keyword(s):  
Maintenance Therapy ◽  
Steroid Therapy ◽  
Autoimmune Pancreatitis ◽  
Elevated Serum ◽  
Oral Prednisolone ◽  
Bile Duct Stenosis ◽  
Glucose Levels ◽  
First Case ◽  
Serum Igg4 ◽  
Duct Stenosis

The first case that led researchers to put forward a new concept of autoimmune pancreatitis (AIP) was treated with steroids by gastroenterologists in Tokyo Women’s Medical University. It is important to differentiate AIP from pancreatic cancer before treatment with steroids is started. Today, steroids are standard therapy for AIP worldwide. In the Japanese consensus guidelines, steroid therapy is indicated for symptomatic AIP. After management of glucose levels and obstructive jaundice, oral prednisolone is initiated at 0.6 mg/kg/day for 2–4 weeks and is gradually tapered to a maintenance dose of 2.5–5 mg/day over 2-3 months. To prevent relapse, maintenance therapy with low-dose prednisolone is used. For relapsed AIP, readministration or increased doses of steroids are effective. The presence of proximal bile duct stenosis and elevated serum IgG4 levels may be predictive of relapse of AIP. It is necessary to verify the validity of the Japanese regimen of steroid therapy for AIP. The necessity, drugs, and duration of maintenance therapy for AIP need to be clarified by prospective studies.

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