Is the Mayo model for predicting survival useful after the introduction of ursodeoxycholic acid treatment for primary biliary cirrhosis

Kilmurry, M.R.

doi:10.1053/jhep.1996.v23.pm0008621147

Cited by 22 publications

(20 citation statements)

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“…Even with total bilirubin levels well within the normal range, the data presented here indicate that direct bilirubin levels may be significantly elevated. Although many guidelines and established risk criteria include total bilirubin, lower thresholds within the normal range may need to be considered for clinical use 4,6,9‐17 . This is further exemplified by the inclusion of total bilirubin as a continuous variable within the UK‐PBC risk score and Global PBC risk score, suggesting that lower total bilirubin levels within any range contribute to a lower risk of liver‐related/all‐cause mortality and liver transplantation 9,16 .…”

Section: Discussionmentioning

confidence: 99%

“…The Global PBC Study Group similarly established that rising total bilirubin levels, even within the normal range, are predictive of the 5‐year risk of liver transplantation or death 8 . In fact, multiple guidelines, response criteria and risk score calculators used for PBC agree that the stabilization or reduction in total bilirubin levels corresponds to reduced risk for disease progression, highlighting the need for PBC treatment options that can stabilize or reduce bilirubin levels in patients with PBC 4,6,9‐17 …”

Section: Introductionmentioning

confidence: 99%

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Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis

Parés

Shiffman

Vargas

et al. 2020

Liver International

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Background & Aims: Total bilirubin is a predictor of survival in primary biliary cholangitis, with the main elevated component being direct bilirubin. The purpose of this post hoc analysis was to assess the efficacy and safety of obeticholic acid across quartiles of varying baseline levels of direct bilirubin in the phase 3, randomized, placebo-controlled Primary Biliary Cholangitis Obeticholic Acid International Study of Efficacy. Methods: This analysis assessed patients on the basis of their baseline direct bilirubin level (divided by quartile). Biochemistry and safety outcomes were evaluated within each quartile over time.Results: In the quartile with the highest baseline direct bilirubin (>5.47 µmol/L), there was a significant reduction in both direct and total bilirubin at Month 12 compared with placebo. Least squares mean (standard error) change from baseline in direct bilirubin at Month 12 was 4.17 (1.42) µmol/L for placebo, −3.48 (1.63) µmol/L for obeticholic acid 5-10 mg and −3.66 (1.51) µmol/L for obeticholic acid 10 mg (P < .0001, obeticholic acid vs placebo); the corresponding values for total bilirubin at Month 12 were 4.38 (1.55) µmol/L for placebo, −4.53 (1.83) µmol/L for obeticholic acid 5-10 mg and −5.06 (1.64) µmol/L for obeticholic acid 10 mg (P < .0001, obeticholic acid vs placebo). Conclusions:Obeticholic acid treatment was associated with significant reductions in total and direct bilirubin, particularly in patients with high baseline direct bilirubin.Because raised direct bilirubin levels, even within the normal range, are predictive of survival in primary biliary cholangitis, these results suggest substantial benefits of obeticholic acid in at-risk patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis

Parés

Shiffman

Vargas

et al. 2020

Liver International

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“…Post-transplantation outcome correlates well with the level of serum bilirubin; optimal post-transplantation survival requires the bilirubin level to be less than 180 mmol/l at the time of transplantation. Although treatment with UDCA reduces the serum bilirubin level, it does not alter the validity of the bilirubin level or the Mayo risk score in assessing prognosis [79].…”

Section: Natural Historymentioning

confidence: 96%

Primary biliary cirrhosis: lessons learned from an organic-specific disease

Nishio

Keeffe

Gershwin³

2001

Clinical and Experimental Medicine

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Primary biliary cirrhosis is an autoimmune liver disease that predominantly affects women and is characterized by chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and subsequent fibrosis. The serological hallmark is the presence of antimitochondrial antibodies, which are found in 95% of patients. These antibodies are directed against the 2-oxo-acid dehydrogenase complexes located on the inner membrane of mitochondria. Although the role of antimitochondrial antibodies in the pathogenesis is unknown, the presence of antibodies has allowed detailed immunological definition of the antigenic epitopes, the autoantibodies, and the T-cell response. Theories have been proposed regarding the mechanism of immune-mediated bile duct damage in primary biliary cirrhosis, including the possible role of T-cell-mediated cytotoxicity and molecular mimicry. Primary biliary cirrhosis is usually diagnosed based on the triad of elevated alkaline phosphatase, antimitochondrial antibodies, and characteristic histological changes on liver biopsy. Biochemical liver abnormalities are consistent with the presence of cholestasis and include an elevation of both serum alkaline phosphatase and gamma-glutamyl transpeptidase, with or without elevation of aminotransferase levels. Ursodeoxycholic acid, a dihydroxy bile acid, appears to be the only effective therapy in preventing or delaying the need for liver transplantation. However, a number of patients receiving ursodeoxycholic acid still develop progressive disease and require transplantation; at present, liver transplantation is the only effective therapy for end-stage primary biliary cirrhosis.

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“…The best validated model is that developed by the Mayo Clinic, but it tends to overestimate the probability of survival for patients with portal hypertensive bleeding. Treatment with UDCA does not appear to compromise the validity of the serum bilirubin or the Mayo model [49].…”

Section: Natural Historymentioning

confidence: 96%

Biliary tract inflammatory disorders: Primary sclerosing cholangitis and primary biliary cirrhosis

Franco

Saeian

1999

Curr Gastroenterol Rep

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Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with end-stage liver disease and yields excellent long-term survival in both groups.

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Is the Mayo model for predicting survival useful after the introduction of ursodeoxycholic acid treatment for primary biliary cirrhosis

Cited by 22 publications

References 0 publications

Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis

Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis

Primary biliary cirrhosis: lessons learned from an organic-specific disease

Biliary tract inflammatory disorders: Primary sclerosing cholangitis and primary biliary cirrhosis

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