scholarly journals IgG4 Related Sclerosing Cholangitis

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
D. Joshi ◽  
G. J. M. Webster
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Differential Diagnoses ◽  
Disease Entity ◽  
Future Directions ◽  
Related Disease ◽  
Management Algorithm ◽  
Igg4 Related Disease

IgG4 related disease (IgG4-RD) is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP). In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC) and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.

scholarly journals Biliary Involvement in Type 2 Autoimmune Pancreatitis

2019 ◽  
Vol 13 (1) ◽  
pp. 200-206 ◽  
Author(s):  
Diana Ollo ◽  
Sylvain Terraz ◽  
Gregoire Arnoux ◽  
Giacomo Puppa ◽  
Jean-Louis Frossard ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Hepatic Duct ◽  
Intrahepatic Bile Duct ◽  
Rare Condition ◽  
Left Hepatic Duct ◽  
Mr Cholangiography ◽  
Igg4 Related Disease

Autoimmune pancreatitis (AIP) is a rare condition classified in 2 subtypes. Their distinction relies on a combination of clinical, serological, morphological and histological features. Type 1 is a pancreatic manifestation of IgG4-related disease characterized by multiorgan infiltration by IgG4 plasmocytes. In this condition, hepatobiliary infiltration is frequent and often mimics cholangiocarcinoma or primary sclerosing cholangitis. On the other hand, type 2 is commonly limited to the pancreas. Herein, we describe the case of a patient who presented a type 2 AIP associated with cholangiopathy, a condition not described in the established criteria. He first developed a pancreatitis identified as type 2 by the typical histopathological features and lack of IgG4 in the serum and tissue. Despite a good clinical response to steroids, cholestasis persisted, identified by MR cholangiography as a stricture of the left hepatic duct with dilatation of the intrahepatic bile duct in segments 2 and 3. Biliary cytology was negative. Evolution was favorable but after steroid tapering a few months later, the patient suffered from recurrence of the pancreatitis as well as progression of biliary attempt, suspicious for cholangiocarcinoma. As the investigations again ruled out neoplastic infiltration or primary sclerosing cholangitis, azathioprine was initiated with resolution of both pancreatic and biliary attempts.

Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings

2001 ◽  
Vol 11 (8) ◽  
pp. 1401-1404 ◽  
Author(s):  
Isabel Eerens ◽  
Dirk Vanbeckevoort ◽  
Werner Vansteenbergen ◽  
Lieven Van Hoe
Keyword(s):  
Mr Imaging ◽  
Primary Sclerosing Cholangitis ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Imaging Findings

Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease

2011 ◽  
Vol 46 (3) ◽  
pp. 277-288 ◽  
Author(s):  
Kazuichi Okazaki ◽  
Kazushige Uchida ◽  
Masanori Koyabu ◽  
Hideaki Miyoshi ◽  
Makoto Takaoka
Keyword(s):  
Autoimmune Pancreatitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Recent Advances

Recurrent Primary Sclerosing Cholangitis: Current Understanding, Management, and Future Directions

2021 ◽  
Author(s):  
Kristel K. Leung ◽  
Maya Deeb ◽  
Sandra E. Fischer ◽  
Aliya Gulamhusein
Keyword(s):  
Risk Factors ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Large Scale ◽  
Related Factors ◽  
Future Directions ◽  
History Of ◽  
Inflammatory Bowel ◽  
Specific Factors ◽  
Associated Risk Factors

AbstractPatients with primary sclerosing cholangitis (PSC) constitute 5 to 15% of patients listed for liver transplantation worldwide. Although post-transplant outcomes are favorable, recurrent PSC (rPSC) occurs in an important subset of patients, with higher prevalence rates reported with increasing time from transplant. Given its association with poor graft outcomes and risk of retransplant, effort has been made to understand rPSC, its pathophysiology, and risk factors. This review covers these facets of rPSC and focuses on implicated risk factors including pretransplant recipient characteristics, inflammatory bowel-disease-related factors, and donor-specific and transplant-specific factors. Confirming a diagnosis of rPSC requires thoughtful consideration of alternative etiologies so as to ensure confidence in diagnosis, management, subsequent risk assessment, and counseling for patients. Unfortunately, no cure exists for rPSC; however, future large-scale efforts are underway to better characterize the natural history of rPSC and its associated risk factors with hopes of identifying potential key targets for novel therapies.

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