Primary sclerosing cholangitis

Angulo, Paul; Lindor, Keith D.

doi:10.1002/hep.510300101

Cited by 235 publications

(154 citation statements)

References 84 publications

(104 reference statements)

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“…1 No effective medical therapy aimed at preventing disease progression currently is available and the disease usually leads to biliary cirrhosis and liver failure. 2 Liver transplantation remains the only life-extending therapeutic alternative for patients with end-stage PSC.…”

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confidence: 99%

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

Feldstein¹

2003

Hepatology

229

214

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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.)

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confidence: 99%

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

Feldstein¹

2003

Hepatology

229

214

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“…1 Approximately 80% of patients have concomitant inflammatory bowel disease, most commonly ulcerative colitis. The strong association between PSC and inflammatory bowel disease has been speculated to cause increased translocation of colonic bacteria and endotoxins, as well as enhanced absorption of toxic bile acids to the liver via the portal vein.…”

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confidence: 99%

“…The strong association between PSC and inflammatory bowel disease has been speculated to cause increased translocation of colonic bacteria and endotoxins, as well as enhanced absorption of toxic bile acids to the liver via the portal vein. 1,2 This may lead to activation of Kupffer cells in the liver, with consequent overproduction of tumor necrosis factor and immunoactivation of biliary epithelial cells in genetically susceptible patients. 2,3 A close genetic association of PSC with HLA-DR3, DQ2 has been reported 4 and is described in other autoimmune liver diseases, as well.…”

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confidence: 99%

Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: A randomized placebo-controlled trial

et al. 2004

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No effective medical therapy is currently available for primary sclerosing cholangitis (PSC). Ursodeoxycholic acid (UDCA) improves liver enzymes, but its effect on liver histology is controversial. Metronidazole (MTZ) prevents PSC-like liver damage in animal models and reduces intestinal permeability. We recruited 80 patients with PSC into a randomized placebo-controlled study to evaluate the effect of UDCA and MTZ (UDCA/MTZ) compared with UDCA/placebo on the progression of PSC. Patients (41 UDCA/placebo and 39 UDCA/ MTZ) were followed every third month. Assessment of liver function test, histological stage and grade, and cholangiography (via ERCP) at baseline showed no differences between the groups. After 36 months, serum aminotransferases ␥-glutamyltransferase, and alkaline phosphatase (ALP) decreased markedly in both groups, serum ALP more significantly in the UDCA/MTZ group (؊337 ؎ 54 U/L, P < .05) compared with the UDCA/placebo group. The New Mayo Risk Score decreased markedly only in the UDCA/MTZ group (؊0.50 ؎ 0.13, P < .01). The number of patients with improvement of stage (P < .05) and grade (P < .05) was higher in the combination group. ERCP findings showed no progression or improvement in 77% and 68% of patients on UDCA/MTZ and UDCA/placebo, respectively. In conclusion, combining MTZ with UDCA in PSC improved serum ALP levels and New Mayo Risk Score, but no statistically significant effect on disease progression as assessed via liver histology or ERCP was seen. Long-term studies using a higher dose of UDCA combined with MTZ in larger patient populations are indicated. (HEPATOLOGY 2004;40:1379 -1386

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“…Inflammatory bowel disease is associated with extra-intestinal manifestations in a significant proportion of patients [1][2][3]. The interaction between inflammatory bowel disease (IBD) and hepatobiliary manifestations represents a classic example of liver-gut interaction.…”

Section: Introductionmentioning

confidence: 99%

Sclerosing Cholangitis with Ulcerative Colitis after Bariatric Surgery

Fayadh¹

2017

J Gastrointest Dig Syst

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Introduction: 48 years old Iraqi female presented in 2012 with 4 months upper abdominal pain with elevation of the liver enzymes and arthralgia, 1 year after sleeve gastrectomy for obesity with 25 kg weight loss. Labeled as auto immune hepatitis and was started on prednisolone 20 mg and azathioprine 100 mg, attended our center for second opinion. Her past history was impressive for gestational diabetes and ulcerative colitis diagnosed before 15 years and treated for 1 year with remission. Strong family history of diabetes including father, mother, brothers, and her uncle has ulcerative colitis.

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Primary sclerosing cholangitis

Cited by 235 publications

References 84 publications

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

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

Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis: A randomized placebo-controlled trial

Sclerosing Cholangitis with Ulcerative Colitis after Bariatric Surgery

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