Portal Hypertension and Primary Biliary Cirrhosis: Effect of Long-Term Ursodeoxycholic Acid Treatment

Huet, Pierre‐Michel; Vincent, Catherine; Deslaurier, Julie; Côté, Jean; Matsutami, Shoichi; Boileau, Robert; Kerckvoorde, Jacline Huet-Van

doi:10.1053/j.gastro.2008.07.019

Cited by 77 publications

(66 citation statements)

References 34 publications

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“…29 In this study, significant differences in survival were noted between patients classified at baseline as having PHG < 6 mm Hg, PHG 6-12 mm Hg, and PHG > 12 mm Hg. 29 Higher PHG at baseline was associated with decreased survival. Furthermore, improvement of PHG in response to UDCA was associated with survival free of liver transplantation.…”

Section: Portal Hypertensionmentioning

confidence: 59%

“…Natural history studies have identified numerous markers of progressive disease in PBC, including but not limited to: response to UDCA, [15][16][17][18] histology (degree of interface hepatitis and features of overlap with autoimmune hepatitis), 23,24 biochemical markers (serum bilirubin, albumin and prothrombin time), 28 presence and/or degree of portal hypertension, 29 certain genetic polymorphisms (apolipoprotein A, tumor necrosis factor [TNF]-alpha), specific autoantibodies (anti-gp210, anti-promyelocytic leukemia protein [PML], anti-sp100, anti-centromere), 30,31 and serum markers of fibrosis (hyaluronic acid, procollagen III, tissue inhibitor metalloproteinase). 32 Models using time-fixed Cox proportional hazards regression analysis have been developed to predict survival in PBC.…”

Section: Stratification Of Subjectsmentioning

confidence: 99%

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American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

et al. 2010

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PreambleA group of multidisciplinary experts on primary biliary cirrhosis (PBC) and its complications convened on May 31, 2009, under the aegis of the American Association for the Study of Liver Diseases (AASLD) in order to identify the most appropriate design and endpoints for clinical trials of PBC based on current evidence and expert experience. The natural history of PBC was reviewed as well as current therapies. The current approaches to evaluating therapies for disease progression and symptoms as priorities were discussed. Appropriate aspects of trial design including entry criteria, study duration, and appropriate handling of issues such as stratification of subjects and use of ursodeoxycholic acid (UDCA), were identified and discussed. After a full day of presentations, in a consensus manner, appropriate endpoints for clinical efficacy trials regarding PBC and its complications were agreed upon and are reported in this summary.

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Section: Portal Hypertensionmentioning

confidence: 59%

Section: Stratification Of Subjectsmentioning

confidence: 99%

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

et al. 2010

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“…This vasculopathy has been postulated to play a role in the pathogenesis of PBC [44] and of PBC-associated portal hypertension [45,46]. Thus, aPL may not only be an epiphenomenon of PBC but they may also contribute to its pathogenesis [32].…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Liver Diseases and Antiphospholipid Antibodies Positivity: a Meta-analysis of Literature Studies

Ambrosino

Lupoli

Spadarella

et al. 2015

JGLD

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Background & Aims: Several studies reported an association between autoimmune liver diseases (AiLD) and antiphospholipid antibodies (aPL) positivity. We performed a meta-analysis of studies evaluating the association of primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH), and primary sclerosing cholangitis (PSC) with aPL positivity and with aPL-related thrombotic events.Methods: Studies evaluating the association of AiLD with aPL (anticardiolipin [aCL], anti-β2 glycoprotein-I [anti-β2GPI], lupus anticoagulant [LA] antibodies) and with aPL-related thrombotic complications were systematically searched in the PubMed, Web of Science, Scopus and EMBASE databases.Results: A total of 10 studies (750 patients with AiLD and 1,244 healthy controls) were included in the analysis on the prevalence of aPL and showed that AiLD are significantly associated with the presence of aCL and anti-β2GPI. The association with aCL positivity was consistently confirmed in PBC (OR: 13.93, 95%CI: 4.69-41.38), AIH (OR: 23.50, 95%CI: 4.28-129.13), and PSC (OR: 18.21, 95%CI: 7.05-47.08). Similarly, anti-β2GPI were found more frequently in PBC (OR: 25.10, 95%CI: 4.77-132.11), AIH (OR: 48.57, 95%CI: 11.07-213.09), and PSC (OR: 36.30, 95%CI: 6.55-201.31). These findings are confirmed when separately analyzing IgM, IgG, and IgA directed against phospholipids. Two of the 10 included articles and 1 further study (67 cases and 75 controls) showed a trend - not achieving statistical significance - towards a higher prevalence of thrombotic complications in AIH patients with aPL as compared to those with only AIH (OR: 1.67, 95%CI: 0.46-6.05).Conclusion: PBC, AIH, and PSC are significantly associated with aPL positivity. The association with aPL-related thrombotic complications should be further studied.

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“…An increased portal pressure defined as the difference between the wedge and free pressure in the hepatic veins is frequently found in PBC. A reliable predictor of survival is the stability or improvement of this pressure gradient [214,215] . Cirrhosis is present only at stage 4, but variceal hemorrhage has been described early in the disease due to early development of nodular regenerative hyperplasia [216] .…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Today there is a more or less general agreement that a biochemical response to a dose of 13-15 mg/kg per day of UDCA delays progression of disease in the majority of patients. Major changes in the natural history of PBC have been achieved, mainly resulting in decreased rates of liver transplantation and prolonged survival due to earlier diagnosis of the disease and hence timely intervention with UDCA [214,262,271,281,286,287] . An increasing use of UDCA is accompanied by a similar decrease in the number of patients transplanted [288,289] .…”

Section: Treatment Of Pbcmentioning

confidence: 99%

Primary biliary cirrhosis: From bench to bedside

Notas

2015

WJGPT

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Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient's genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed. Core tip: Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis of largely unknown pathophysiology. We examined the epidemiological and geoepidemiological data as well as the pathogenetic aspects of PBC. A novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.

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Portal Hypertension and Primary Biliary Cirrhosis: Effect of Long-Term Ursodeoxycholic Acid Treatment

Cited by 77 publications

References 34 publications

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

Autoimmune Liver Diseases and Antiphospholipid Antibodies Positivity: a Meta-analysis of Literature Studies

Primary biliary cirrhosis: From bench to bedside

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