Primary sclerosing cholangitis: findings on cholangiography and pancreatography.

MacCarty, Robert L.; LaRusso, N. F.; Wiesner, R H; Ludwig, Jürgen

doi:10.1148/radiology.149.1.6412283

Cited by 317 publications

(145 citation statements)

References 0 publications

Supporting

Mentioning

129

Contrasting

Unclassified

Order By: Relevance

“…These patients had their first medical evaluation for their liver disease at our institution during the period from January 1, 1980, to December 31, 1999. The diagnosis of PSC was established unequivocally based on typical cholangiographic features 8 shown by endoscopic retrograde cholangiography in 50 patients and by percutaneous transhepatic cholangiography in 2 patients. All cholangiography images were interpreted by independent radiologists with experience in biliary tree imaging.…”

Section: Methodsmentioning

confidence: 99%

Primary sclerosing cholangitis in children: A long-term follow-up study

Feldstein¹

2003

Hepatology

229

214

View full text Add to dashboard Cite

Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long-term outcome of children with PSC. Fifty-two children with cholangiography-proven PSC (34 boys and 18 girls; mean age 13.8 ؎ 4.2 years; range, 1.5-19.6 years) who were seen at our institution over a 20-year period were followed-up for up to 16.7 years. Two thirds presented with symptoms and/or signs of PSC and 81% had concomitant inflammatory bowel disease (IBD). Twenty-five percent had total alkaline phosphatase activity within the normal range for the age group, but all of them had elevated ␥-glutamyl transpeptidase levels. Autoimmune hepatitis overlapping with PSC was present in 35% of children. A positive but transient clinical and/or biochemical response occurred under therapy with ursodeoxycholic acid, alone or in combination with immunosuppressive medications. During follow-up, 11 children underwent liver transplantation for end-stage PSC and 1 child died. The median (50%) survival free of liver transplantation was 12.7 years. Compared with an age-and gender-matched U.S. population, survival was significantly shorter in children with PSC (P < .001). In a Cox regression model, lower platelet count, splenomegaly, and older age were associated with shorter survival. Presence of autoimmune hepatitis overlapping with PSC (P ‫؍‬ .2) or medical therapy (P ‫؍‬ .2) did not affect survival. In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long-term outcome. (HEPATOLOGY 2003;38:210-217.)

show abstract

Section: Methodsmentioning

confidence: 99%

Primary sclerosing cholangitis in children: A long-term follow-up study

Feldstein¹

2003

Hepatology

229

214

View full text Add to dashboard Cite

show abstract

“…2,7,30,31 The definition of recurrent primary sclerosing cholangitis usually is based on published criteria 3 including biopsy and cholangiographic findings. 4,5 In selected cases, partial resection of liver graft tissue that has limited biliary injury may be possible to prevent complete loss of the transplanted liver and revision transplant. 32 In addition, revision transplant and death were primary endpoints.…”

Section: Discussionmentioning

confidence: 99%

“…3 Disease-free survival ends with histologic proof of recurrent primary sclerosing cholangitis, with fibrous cholangitis, fibrous and stenosing lesions of the bile ducts with or without histologic proof of recurrent primary sclerosing cholangitis, with biliary fibrosis, or cirrhosis (Table 1). In addition, cholangiographic findings on endoscopic retrograde cholangiography 4 or magnetic resonance cholangiography 5 have been used for diagnosis of recurrent primary sclerosing cholangitis.…”

Section: Introductionmentioning

confidence: 99%

Survival Without Biliary Complications After Liver Transplant for Primary Sclerosing Cholangitis

Mogl

Albert²,

Pascher³

et al. 2013

Exp Clin Transplant

View full text Add to dashboard Cite

Objectives: Patients who have a liver transplant for primary sclerosing cholangitis may develop recurrent disease and biliary complications, organ loss necessitating revision liver transplant, or death. We evaluated long-term outcomes in patients who had liver transplant for primary sclerosing cholangitis. Materials and Methods: In 71 patients who had a liver transplant for end-stage liver disease because of primary sclerosing cholangitis, a retrospective review was done to evaluate biliary complicationfree survival, transplanted organ survival, and death. Human leukocyte antigen typing and matching were reviewed. Results: There were 39 patients (55%) who had biliary complications, loss of the liver transplant, or death at a mean 12.1 years after transplant. The 5-and 10-year event-free survival reached 74.6% and 45% (53 patients after 5 years, and 32 patients after 10 years). Male sex of transplant recipients was a significant risk factor for biliary complications, revision liver transplant, or death. Most patients had inflammatory bowel disease, primarily ulcerative colitis. The human leukocyte antigen profile or number of mismatches had no effect on complication-free survival. Conclusions: Biliary complications, revision liver transplant, and death are a useful combined primary endpoint for recurrent primary sclerosing cholangitis after liver transplant.

show abstract

“…Approximately 25% of patients with small-duct PSC progress to large-duct PSC, but usually does not develop into cholangiocarcinoma unless it progresses to large-duct PSC (Bjornsson, Olsson et al, 2008). The gold standard for diagnosis is endoscopic retrograde cholangiopancreatography (ERCP) and typical radiographic findings include multifocal strictures and dilatation of the intraand/or extra-hepatic biliary tracts, producing a "beaded pattern" (Lee & Kaplan, 1995;Maccarty et al, 1983). Recently, magnetic resonance cholangiopancreatography (MRCP) has emerged as a non-invasive alternative in diagnosing suspected PSC (Chapman, Fevery et al, 2010).…”

Section: Primary Sclerosing Cholangitis (Psc)mentioning

confidence: 99%