scholarly journals Waitlist survival of patients with primary sclerosing cholangitis in the model for end-stage liver disease era

2011 ◽  
Vol 17 (11) ◽  
pp. 1355-1363 ◽  
Author(s):  
David Goldberg ◽  
Benjamin French ◽  
Arwin Thomasson ◽  
K. Rajender Reddy ◽  
Scott D. Halpern
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
End Stage Liver Disease ◽  
End Stage

scholarly journals Outcome stagnation of liver transplantation for primary sclerosing cholangitis in the Model for End-Stage Liver Disease era

2014 ◽  
Vol 399 (8) ◽  
pp. 1021-1029 ◽  
Author(s):  
Johannes Klose ◽  
Michelle A. Klose ◽  
Courtney Metz ◽  
Frank Lehner ◽  
Michael P. Manns ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
End Stage Liver Disease ◽  
End Stage

Case 14.4

2014 ◽  
pp. 681-682
Author(s):  
Christine U. Lee ◽  
James F. Glockner
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Vertebral Body ◽  
Signal Intensity ◽  
Sclerosing Cholangitis ◽  
Cirrhotic Liver ◽  
Weighted Image ◽  
End Stage Liver Disease ◽  
Lumbar Vertebral Body ◽  
End Stage

80-year-old man with primary sclerosing cholangitis and end-stage liver disease Axial fat-suppressed FSE T2-weighted image (Figure 14.4.1) demonstrates increased signal intensity in a lumbar vertebral body. There is corresponding enhancement on a postgadolinium 2D SPGR image (Figure 14.4.2). Note also abdominal ascites, peritoneal enhancement, and a cirrhotic liver. Coronal and sagittal reformatted views (...

scholarly journals Primary sclerosing cholangitis

2008 ◽  
Vol 22 (8) ◽  
pp. 689-698 ◽  
Author(s):  
Marina G Silveira ◽  
Keith D Lindor
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Serum Alkaline Phosphatase ◽  
Cholestatic Liver Disease ◽  
Biliary Strictures ◽  
Vitamin Deficiencies ◽  
Metabolic Bone ◽  
High Doses ◽  
End Stage

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day) is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

2017 ◽  
Vol 37 (04) ◽  
pp. 305-313 ◽  
Author(s):  
Cynthia Levy ◽  
Eric Martin
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Graft Survival ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Immune Mediated ◽  
End Stage ◽  
Patient And Graft Survival

AbstractPrimary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

scholarly journals Management of primary sclerosing cholangitis and its complications: an algorithmic approach

2020 ◽  
Author(s):  
Michal Prokopič ◽  
Ulrich Beuers
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Treatment Options ◽  
Diagnostic Procedures ◽  
Disease Recurrence ◽  
Cholestatic Liver Disease ◽  
Diagnostic Challenge ◽  
Algorithmic Approach ◽  
End Stage

AbstractPrimary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.

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