The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis

Corpechot, Christophe; Carrat, Fabrice; Bahr, Abbas; Chrétien, Yves; Poupon, R; Poupon, Raoul

doi:10.1053/j.gastro.2004.11.009

Cited by 365 publications

(237 citation statements)

References 36 publications

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“…Dotted lines for each symptom domain represent the cutoff for clinically significant symptoms (defined as mean value for normal subjects 12 SD). Solid line in (E) and (F) represents the empirical cutoffs used in clinical practice for ESS (10) and OGS (4). Similarity between the empirical and defined cutoffs established in the study suggests that the control population is representative of community norms.…”

Section: Resultsmentioning

confidence: 71%

Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study

et al. 2013

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,4 and the UK-PBC Consortium Primary biliary cirrhosis (PBC) has a complex clinical phenotype, with debate about the extent and specificity of frequently described systemic symptoms such as fatigue. The aim of this study was to use a national patient cohort of 2,353 patients recruited from all clinical centers in the UK to explore the impact of disease on perceived life quality. Clinical data regarding diagnosis, therapy, and biochemical status were collected and have been reported previously. Detailed symptom phenotyping using recognized and validated symptom assessment tools including the PBC-40 was also undertaken and is reported here. Perception of poor quality of life and impaired health status was common in PBC patients (35% and 46%, respectively) and more common than in an age-matched and sex-matched community control group (6% and 15%, P < 0.0001 for both). Fatigue and symptoms of social dysfunction were associated with impaired perceived quality of life using multivariate analysis. Fatigue was the symptom with the greatest impact. Depression was a significant factor, but appeared to be a manifestation of complex symptom burden rather than a primary event. Fatigue had its greatest impact on perceived quality of life when accompanied by symptoms of social dysfunction, suggesting that maintenance of social networks is critical for minimizing the impact of fatigue. Conclusion: The symptom burden in PBC, which is unrelated to disease severity or ursodeoxycholic acid response, is significant and complex and results in significant quality of life deficit. The complexity of symptom burden, and its lack of relation to disease severity and treatment response, suggest that specific approaches to symptom management are warranted that address both symptom biology and social impact. (HEPATOLOGY 2013;58:273-283)

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Section: Resultsmentioning

confidence: 71%

Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study

et al. 2013

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“…Treatment with UDCA has been shown to significantly lower the serum levels of bilirubin, ALP, GGT, IgM and total cholesterol (143,144) . Studies also demonstrated that treatment with UDCA slows histological progression, delays development of esophageal varices, and normalizes survival free of LT for patients who begin treatment at earlier histological stages (145)(146)(147)(148) . Unfortunately, UDCA does not improve fatigue or pruritus.…”

Section: Treatment With Ursodeoxycholic Acidmentioning

confidence: 97%

“…These patients have poorer prognosis (147)(148)(149) . Ductopenia, advanced histological stages (stages III and IV) and moderate-severe interface hepatitis, bilirubin > 1 mg/dL, low serum albumin levels, portal hypertension and the Mayo risk score are all pre-treatment factors associated with poorer response to UDCA and poorer overall prognosis (146)(147)(148)(149)151,155) . As PBC characteristically affects women older than 40 years of age, pregnancy is not common after the diagnosis.…”

Section: Treatment With Ursodeoxycholic Acidmentioning

confidence: 99%

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

Bittencourt

Cançado

Couto

et al. 2015

Arq. Gastroenterol.

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-In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

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“…In many cases, the disease is detected at an early stage and treatment with ursodeoxycholic acid (UDCA) improves biochemistry, impedes hepatic fibrosis, and can restore normal life expectancy 3, 4, 5. However, in a substantial proportion of patients, the response to treatment with UDCA is inadequate.…”

mentioning

confidence: 99%

Clinical application of the GLOBE and United Kingdom‐primary biliary cholangitis risk scores in a trial cohort of patients with primary biliary cholangitis

Carbone

Harms

Lammers

et al. 2018

Hepatology Communications

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The GLOBAL Primary Biliary Cholangitis (PBC) Study Group and United Kingdom‐PBC (UK‐PBC) Consortium have demonstrated that dichotomous response criteria are not as accurate as continuous equations at predicting mortality or liver transplantation in PBC. The aim of this analysis was to assess the clinical utility of the GLOBE and UK‐PBC risk scores using data from POISE, a phase 3 trial investigating obeticholic acid (OCA) in patients with PBC. Data (N = 216) at baseline and month 12 were used to calculate the GLOBE and UK‐PBC risk scores to assess the projected change in risk with OCA versus placebo. Additionally, the benefit of OCA was assessed in patients not meeting the POISE primary endpoint. Both the GLOBE and UK‐PBC risk scores predicted a significant reduction in long‐term risk of death and liver transplantation after OCA treatment (P < 0.0001). The differences in the relative risk reduction from baseline in the 10‐year event risk after 1 year for OCA 10 mg versus placebo was 26% (GLOBE) and 37% (UK‐PBC). The scores also predicted a significantly decreased risk in patients treated with OCA who did not meet POISE response criteria after 1 year of treatment compared to an increased risk with placebo (P < 0.0001). Conclusion: This analysis demonstrates the use of the GLOBE and UK‐PBC risk scores to assess risk reduction of a cohort treated with OCA. While validation of this risk reduction in studies with clinical outcomes is needed, this study highlights the potential use of these scores in individualizing risk prediction in PBC both in clinical practice and therapeutic trials. (Hepatology Communications 2018;2:683‐692)

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The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis

Cited by 365 publications

References 36 publications

Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study

Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

Clinical application of the GLOBE and United Kingdom‐primary biliary cholangitis risk scores in a trial cohort of patients with primary biliary cholangitis

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