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.

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.

A Rare Cause of Distal Bile Duct Stenosis

2007 ◽  
Vol 38 (2-4) ◽  
pp. 157-159 ◽  
Author(s):  
F. Stoeckler ◽  
E. Hagmüller ◽  
H.-J. Rumpelt ◽  
U. Weickert
Keyword(s):  
Bile Duct ◽  
Distal Bile Duct ◽  
Bile Duct Stenosis ◽  
Duct Stenosis

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 Diagnosis of Cancer Spread Using Percutaneous Transhepatic Biliary Cholangioscopy-guided Ultrasonography for Malignant Bile Duct Stenosis

2001 ◽  
Vol 7 (3-4) ◽  
pp. 159-164 ◽  
Author(s):  
Hirokazu Inoue
Keyword(s):  
Bile Duct ◽  
Extrahepatic Bile Duct ◽  
Biliary Duct ◽  
Wall Thickening ◽  
Papilla Of Vater ◽  
Extrahepatic Bile Duct Cancer ◽  
Bile Duct Stenosis ◽  
Cancer Spread ◽  
Duct Stenosis ◽  
Maximum Minimum

The characteristics of sites of intramural cancer spread were examined by comparing the intraductal ultrasonography (IDUS) and wall thickening findings at sites of intramural cancer spread and non-spread, in patients with malignant bile duct stenosis who had undergone percutaneous transhepatic biliary drainage (PTBD).The subjects were ten patients with extrahepatic bile duct cancer, two with pancreatic cancer, one with cancer of the gallbladder, and one with cancer of the papilla of Vater who underwent preoperative IDUS. From these patients, 50 IDUS slices were examined with a congruent relationship with the histologic section of resected tissue. The maximum thickening, minimum thickening, maximum/minimum thickening ratio, and form factor of the medial and lateral margins of the medial hypoechoic layer were determined using diagnostic imaging, and the results were compared at sites of cancer spread and non-spread.Twelve slices were obtained from the site of stenosis, 14 from sites of cancer spread, and 24 from non-spread sites. The maximum thickening, minimum thickening, and maximum/minimum thickening ratio differed significantly between the sites of spread and the non-spread.The absolute values for wall thickening are useful for diagnosing the presence of intramural spread in patients with malignant biliary duct stenosis.

scholarly journals Fully Covered Self-Expandable Metal Stents for Treatment of Both Benign and Malignant Biliary Disorders

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Ahmed Abdel Samie ◽  
Lorenz Theilmann
Keyword(s):  
Bile Duct ◽  
Endoscopic Sphincterotomy ◽  
Biliary Obstruction ◽  
Therapeutic Option ◽  
Metal Stents ◽  
Distal Bile Duct ◽  
Biliary Strictures ◽  
Bile Duct Stenosis ◽  
Self Expanding Metal Stents ◽  
Duct Stenosis

Transpapillary stents are increasingly being used for biliary strictures, whether benign or malignant. However, there are different stent types and available data is controversial. Recently, completely covered self-expandable metal stents (CSEMSs) have been proposed as an alternative therapeutic option in different biliary indications, including strictures of the distal bile duct, anastomotic stenosis after orthotopic liver transplantation, bile duct leaks, periampullary perforation following endoscopic sphincterotomy (ES), and postsphincterotomy bleeding. Despite the higher costs of these devices, fully covered self-expanding metal stents seem to be a suitable therapeutic option to relief biliary obstruction due to bile duct stenosis, regardless of the underlying cause.

scholarly journals Diagnosis and management of IgG4-related disease

2018 ◽  
Vol 10 (3) ◽  
pp. 275-283 ◽  
Author(s):  
Vinod S Hegade ◽  
Maria B Sheridan ◽  
Matthew T Huggett
Keyword(s):  
Sclerosing Cholangitis ◽  
Current Knowledge ◽  
Evidence Base ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Storiform Fibrosis ◽  
Type 1 Autoimmune Pancreatitis ◽  
Lymphoplasmacytic Infiltration

IgG subclass 4-related disease (IgG4-RD) is a rare but increasingly recognised fibroinflammatory condition known to affect multiple organs. IgG4-RD is characterised by unique histological features of lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. In this review we describe the pancreaticobiliary manifestations of IgG4-RD, with particular emphasis on type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). AIP and IgG4-SC can pose diagnostic challenges to the clinician as they may mimic pancreatic cancer and primary sclerosing cholangitis, respectively. We discuss current knowledge, clinical diagnostic criteria and recent advances and summarise the evidence base for current therapeutic approaches for AIP and IgG4-SC.

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 Outcomes of Endoscopic Ultrasound-Guided Biliary Drainage in a General Hospital for Patients with Endoscopic Retrograde Cholangiopancreatography-Difficult Transpapillary Biliary Drainage

2021 ◽  
Vol 10 (18) ◽  
pp. 4105
Author(s):  
Naosuke Kuraoka ◽  
Satoru Hashimoto ◽  
Shigeru Matsui ◽  
Shuji Terai
Keyword(s):  
Bile Duct ◽  
Endoscopic Ultrasound ◽  
Biliary Drainage ◽  
Duodenal Bulb ◽  
Distal Bile Duct ◽  
Ultrasound Guided ◽  
Difficult Patients ◽  
Stent Patency ◽  
Bile Duct Stenosis ◽  
Duct Stenosis

Endoscopic ultrasound-guided biliary drainage (EUS-BD) has been developed as an alternative treatment for percutaneous transhepatic biliary drainage for patients with bile duct stenosis. At specialized hospitals, the high success rate and effectiveness of EUS-BD as primary drainage has been reported. However, the procedure is highly technical and difficult, and it has not been generally performed. In this study, we retrospectively examined the effectiveness of EUS-BD in ERCP-difficult patients with distal bile duct stenosis. We retrospectively examined 24 consecutive cases in which EUS-BD was performed at our hospital for distal bile duct stenosis from October 2018 to December 2020. EUS-guided choledochoduodenostomy (EUS-CDS) was selected for cases that could be approached from the duodenal bulb, and EUS-HGS was selected for other cases. In the EUS-CDS and EUS-HGS groups, the technical success rates were 83.3% (10/12] and 91.7% (11/12], respectively. An adverse event occurred in one case in the EUS-CDS group, which developed severe biliary peritonitis. The stent patency period was 91 and 101 days in the EUS-CDS and EUS-HGS groups, respectively. EUS-BD for ERCP-difficult patients with distal bile duct stenosis is considered to be an effective alternative for biliary drainage that can be performed not only in specialized hospitals but also in general hospitals.

scholarly journals Endoscopic laser lithotripsy for complicated bile duct stones: is cholangioscopic guidance necessary?

2007 ◽  
Vol 44 (2) ◽  
pp. 137-140 ◽  
Author(s):  
Ralf Jakobs ◽  
Julio C. Pereira-Lima ◽  
Aline W. Schuch ◽  
Lucas F. Pereira-Lima ◽  
Axel Eickhoff ◽  
...  
Keyword(s):  
Bile Duct ◽  
Laser Lithotripsy ◽  
Bile Duct Stones ◽  
Distal Bile Duct ◽  
Laser Guidance ◽  
Endoscopic Papillotomy ◽  
Intrahepatic Stones ◽  
Bile Duct Stenosis ◽  
Extracorporeal Shock Wave ◽  
Fluoroscopic Control

BACKGROUND: Endoscopic papillotomy is successful in more than 95% of the cases of choledocholithiasis. For patients with difficult bile duct stones not responding to mechanical lithotripsy, different methods for stone fragmentation have been developed. AIM: To compare the results of laser lithotripsy with a stone-tissue recognizing system, when guided by fluoroscopy only or by cholangioscopy. METHODS: Between 1992 and 2002 we have treated 89 patients with difficult bile duct stones by endoscopic retrograde cholangiopancreatography and laser lithotripsy. Unsuccessful extracorporeal shock-wave lithotripsy and electrohydraulic were also performed before laser in 35% and 26% of the cases, respectively. RESULTS: Laser was effective in 79.2% of 72 patients guided by cholangioscopy and in 82.4% of 17 cases steered by fluoroscopy. The median number of impulses in the latter was 4,335 and 1,800 with the former technique. Two parameters influenced the manner of laser guidance. In cases of stones situated above a stricture, cholangioscopic control was more effective (64.7% vs. 31.9%). When the stones were in the distal bile duct, fluoroscopic control was more successful. CONCLUSION: In cases of difficult stones in the distal bile duct, laser lithotripsy under fluoroscopic control is very effective and easily performed. Cholangioscopic guidance should be recommended just in cases of intrahepatic stones or in patients with stones situated proximal to a bile duct stenosis. In these cases, cholangioscopy should be performed either endoscopically or percutaneously.

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