Biliary Liver Cirrhosis (Primary Biliary Cirrhosis)

FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.

Also Known As:

Primary Biliary Cirrhosis; Biliary Cirrhosis; Secondary Biliary Cirrhosis; Liver Cirrhosis, Biliary; Biliary Cirrhosis, Primary, 1; Secondary Biliary Cholangitis; Biliary Cholangitides, Primary; Biliary Cholangitis, Primary; Biliary Cholangitis, Secondary; Cholangitides, Primary Biliary; Cholangitis, Primary Biliary; Cholangitis, Secondary Biliary; Cirrhosis, Biliary; Cirrhosis, Secondary Biliary; Liver Cirrhoses, Biliary; Obstructive Liver Cirrhosis; Primary Biliary Cholangitides; Secondary Biliary Cholangitides; Biliary Cirrhosis, Primary; Biliary Cirrhosis, Secondary; Cholangitis, Chronic Nonsuppurative Destructive; Liver Cirrhosis, Obstructive; Primary Biliary Cholangitis

Networked: 5032 relevant articles (251 outcomes, 547 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Liver Diseases (Liver Disease)
2.	Sclerosing Cholangitis
3.	Pruritus (Itching)
4.	Cholestasis
5.	Autoimmune Hepatitis

Experts

1.	Gershwin, M Eric: 192 articles (01/2022 - 01/2002)
2.	Invernizzi, Pietro: 87 articles (01/2022 - 07/2003)
3.	Coppel, Ross L: 61 articles (01/2022 - 01/2002)
4.	Ansari, Aftab A: 59 articles (01/2022 - 01/2002)
5.	Lindor, Keith D: 57 articles (12/2021 - 01/2002)
6.	Leung, Patrick S C: 52 articles (10/2022 - 05/2003)
7.	Selmi, Carlo: 52 articles (01/2022 - 01/2002)
8.	Nakanuma, Yasuni: 51 articles (01/2022 - 01/2002)
9.	Hirschfield, Gideon M: 48 articles (01/2022 - 05/2008)
10.	Tsuneyama, Koichi: 40 articles (01/2022 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Biliary Liver Cirrhosis:

1.	Ursodeoxycholic Acid (Urso)FDA LinkGeneric 11/01/1998 - "A 1-year randomized, double-blind, placebo-controlled trial was carried out in 50 patients with primary biliary cirrhosis, who had already been treated with ursodeoxycholic acid for at least 1 year, but had not achieved complete disease remission. " 12/01/1995 - "Ursodeoxycholic acid (UDCA) administration can obtain marked improvement of primary biliary cirrhosis (PBC). " 11/01/2001 - "The aim of this study was to assess the safety and efficacy of high-dose ursodeoxycholic acid (UDCA, 28-32 mg/kg/day) in patients with primary biliary cirrhosis (PBC) who had shown an incomplete response to the standard dose (13-15 mg/kg/day). " 07/01/2002 - "Improved liver tests and greater biliary enrichment with high dose ursodeoxycholic acid in early stage primary biliary cirrhosis." 06/01/1998 - "Although ursodeoxycholic acid is beneficial in the treatment of primary biliary cirrhosis, it had no measurable effect on the autoimmune conditions coassociated with the disease."
2.	Bile Acids and Salts (Bile Acids)IBA 01/01/1976 - "These studies suggest that serial measurement of serum bile acids may aid in the evaluation of primary biliary cirrhosis." 03/01/2023 - "To determine 15 bile acid metabolic products in human serum by liquid chromatography-tandem mass spectrometry (LC/MS/MS) and value their diagnostic outcome in primary biliary cholangitis (PBC). " 03/01/2023 - "Quantitative Detection of 15 Serum Bile Acid Metabolic Products by LC/MS/MS in the Diagnosis of Primary Biliary Cholangitis." 01/01/2023 - "Breach of tolerance versus burden of bile acids: Resolving the conundrum in the immunopathogenesis and natural history of primary biliary cholangitis." 01/01/2021 - "Background: Increasing data suggests an interaction between bile acids and intestinal microbiota in the pathogenesis of primary biliary cholangitis (PBC). "
3.	BezafibrateIBA 04/01/2002 - "Is bezafibrate histologically effective for primary biliary cirrhosis?" 01/01/2002 - "Investigation into the efficacy of bezafibrate against primary biliary cirrhosis, with histological references from cases receiving long term monotherapy." 06/07/2018 - "A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis." 01/18/2012 - "We need more randomised clinical trials on the effects of bezafibrate on primary biliary cirrhosis with low risks of systematic errors and random errors." 01/18/2012 - "All randomised clinical trials comparing bezafibrate at any dose or regimen in patients with primary biliary cirrhosis with placebo or no intervention, or with another drug. "
4.	obeticholic acidIBA 01/01/2022 - "Obeticholic acid (OCA), a steroidal BA-like FXR agonist, has been shown to improve liver function in patients with primary biliary cholangitis and is approved as second-line treatment for patients with an inadequate response or intolerance to UDCA. " 02/01/2021 - "Long-Term Efficacy and Safety of Obeticholic Acid in Primary Biliary Cholangitis: Responder Analysis of More Than 5 Years of Treatment in the POISE Trial." 02/01/2021 - "Long-Term Efficacy and Safety of Obeticholic Acid in Patients With Primary Biliary Cholangitis: A Demographic Subgroup Analysis of 5-Year Results From the POISE Trial." 01/01/2019 - "The aim of this study was to evaluate the long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis using 3-year interim data from the 5-year open-label extension of the pivotal phase 3 POISE trial. " 01/01/2019 - "Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study."
5.	Colchicine FDA LinkGeneric 08/01/2005 - "Colchicine for primary biliary cirrhosis: a Cochrane Hepato-Biliary Group systematic review of randomized clinical trials." 04/01/1995 - "Colchicine has been observed to reduce mortality in primary biliary cirrhosis in one study. " 07/01/1988 - "We have conducted a double-blind controlled trial of colchicine in patients with primary biliary cirrhosis. " 08/01/1987 - "Few serious side effects were encountered, and colchicine clearly merits long-term study in the treatment of primary biliary cirrhosis." 08/01/1987 - "Colchicine (1 mg/day), or an identical placebo, was given to 64 patients with primary biliary cirrhosis in a double-blind controlled trial. "
6.	Fibric Acids (Fibrates)IBA 09/01/2023 - "Efficacy of fibrates in the treatment of primary biliary cholangitis: a meta-analysis." 01/01/2022 - "Previous studies have shown the effectiveness of fibrates in primary biliary cholangitis. " 09/01/2011 - "No more pilots, a phase III trial of fibrates in primary biliary cirrhosis is long overdue!" 09/01/2017 - "Fibrates for Primary Biliary Cholangitis: What's All the Hype?" 01/01/2016 - "Fibrates appear to improve biochemistry in patients with primary biliary cholangitis (PBC), but it is unclear which factors predict response and whether treatment improves transplant-free survival. "
7.	BilirubinIBA 01/01/2020 - "Total bilirubin is a predictor of survival in primary biliary cholangitis, with the main elevated component being direct bilirubin. " 08/01/2012 - "[A clinical study of bilirubin rise in short-term in patients with primary biliary cirrhosis]." 06/01/2008 - "The new approach is illustrated with primary biliary cirrhosis (PBC) data, aiming to capture nonlinear patterns of serum bilirubin time courses and their relationship with survival time of PBC patients." 07/01/2002 - "Patients had early stage primary biliary cirrhosis as serum bilirubin levels were normal and the Mayo risk score 4.2 +/- 0.5. " 10/01/1999 - "Is serum bilirubin concentration the only valid prognostic marker in primary biliary cirrhosis?"
8.	Alkaline PhosphataseIBA 11/01/1986 - "It is concluded that, in this study, the finding of an antimitochondrial antibody titer greater than or equal to 1/40 is strongly suggestive of primary biliary cirrhosis even in the absence of symptoms and the presence of a normal alkaline phosphatase." 01/01/2022 - "The diagnosis of primary biliary cholangitis (PBC) in patients with seropositive anti-mitochondrial antibody (AMA) but normal alkaline phosphatase (ALP) depends on a liver biopsy. " 01/01/2021 - "Primary biliary cholangitis with normal alkaline phosphatase: A neglected clinical entity challenging current guidelines." 01/01/2018 - "We did logistic regression analysis of pretreatment variables in a discovery cohort of patients in the UK with primary biliary cholangitis to derive the best-fitting model of UDCA response, defined as alkaline phosphatase less than 1·67 times the upper limit of normal (ULN), measured after 12 months of treatment with UDCA. " 07/01/2017 - "Positive antimitochondrial antibody but normal serum alkaline phosphatase levels: Could it be primary biliary cholangitis?"
9.	EnzymesIBA 08/30/2023 - "According to the progressive rise of liver enzymes with a cholestatic pattern in October 2020, a liver biopsy was performed that showed tiny nests of neuroendocrine-like cells with a background of primary biliary cholangitis (PBC). " 07/01/2010 - "A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. " 12/01/2008 - "Clinical phenotype of primary biliary cirrhosis was verified with positive anti-mitochondrial antibody or M2 antibody, demonstration of cholestatic liver enzymes, and staging of liver biopsy. " 09/01/2008 - "Primary biliary cirrhosis (PBC) was diagnosed on the basis of the elevated serum biliary-enzyme levels, strongly positive antimitochondrial antibody titer, and histologic features of the liver-biopsy specimens showed stage-3 PBC. " 07/01/2000 - "Definite primary biliary cirrhosis was defined as a positive antimitochondrial antibody titre > or =1/40, abnormal liver enzymes and compatible/diagnostic histology; probable as the presence of two of these indications. "
10.	Methotrexate (Mexate)FDA LinkGeneric 11/01/1991 - "The data suggest that methotrexate may be effective treatment for precirrhotic primary biliary cirrhosis. " 07/20/2005 - "We do not recommend methotrexate for patients with primary biliary cirrhosis outside randomised trials." 08/01/1999 - "Low-dose methotrexate is ineffective in primary biliary cirrhosis: long-term results of a placebo-controlled trial." 02/01/1996 - "However, prospective, randomized controlled trials will be necessary for definitive evaluation of the effects of methotrexate on the quality of life and survival of patients with primary biliary cirrhosis." 11/01/2010 - "Methotrexate for primary biliary cirrhosis: who is to be trusted?"

Therapies and Procedures

1.	Therapeutics 07/01/1997 - "Unless larger and longer controlled trials with clinical, biochemical and histologic controls show it to be a safe and effective therapy for primary biliary cirrhosis, ursodeoxycholic acid+methotrexate should not be used as a proven and accepted treatment." 08/01/1998 - "In 26 patients with primary biliary cirrhosis stage II, III or IV, therapy showed a trend to improved survival, but this was still significantly worse than the general population. " 01/01/2017 - "Current trials with drugs that target BS toxicity are effective in only about 50%-60% of primary biliary cholangitis patients, with no effective therapy in PSC. " 04/01/1996 - "The close correlation between the improvement in the imbalance of lymphocyte subsets after the therapy and the clinical status suggests that an immunological process may play a role in the effectiveness of therapy in primary biliary cirrhosis." 05/01/2023 - "This study aims to assess the health-related quality of life (HRQoL) in a Dutch population of patients with primary biliary cholangitis (PBC) in relation to the prognosis and need for second line-therapy, based on both objective disease parameters and patients' perspectives. "
2.	Liver Transplantation 07/01/1995 - "A well documented improvement of Raynaud's phenomenon, following successful liver transplantation realized for end-stage primary biliary cirrhosis, using repetitive angiological examination and capillary microscopy is presented." 12/01/1989 - "Liver transplantation is a drastic but successful remedy for complications of abnormal lipid metabolism associated with primary biliary cirrhosis." 04/28/1988 - "Liver transplantation was performed in six of the patients and improved sulfoxidation in five; in the four with primary biliary cirrhosis, sulfoxidation improved from poor to good or intermediate. " 06/01/2003 - "Liver transplantation remains the only effective treatment for end-stage primary biliary cirrhosis (PBC). " 01/01/2001 - "Orthotopic liver transplantation (OLT) is an effective treatment for patients with advanced primary biliary cirrhosis (PBC). "
3.	Transplantation 09/27/2001 - "The prognosis of patients with primary biliary cirrhosis has improved since the introduction of transplantation. " 11/01/2001 - "1. Recurrent primary biliary cirrhosis (PBC) after transplantation is controversial, but most studies support disease recurrence within the graft. " 04/01/2001 - "This study reviews the prevalence of MR imaging abnormalities seen in 21 consecutive patients with primary biliary cirrhosis before transplantation and describes a new MR imaging sign in these patients: the MR imaging periportal halo sign. " 07/01/1999 - "Data on all trial patients referred for transplantation were retrospectively collected from three randomized controlled trials of UDCA in patients with primary biliary cirrhosis (PBC). " 02/01/1999 - "The aim of the study was to use prognostic modelling to determine the optimal timing of transplantation for patients with primary biliary cirrhosis. "
4.	Aftercare (After-Treatment) 07/01/2001 - "T cell repertoire in primary biliary cirrhosis: a common T cell clone and repertoire change after treatment." 01/01/1985 - "Ten patients with primary biliary cirrhosis (PBC) were clinically followed under the conditions of follow-up care. " 12/31/1992 - "We discuss the case of a female patient with breast cancer and primary biliary cirrhosis whose biochemical tests of liver function and the titer of antimitochondrial antibody persistently improved after treatment with 3 cycles of CMF. " 11/01/2009 - "In this long-term follow-up study, nonoperative therapy, biliary cirrhosis, residual stones, and biliary stricture were associated with recurrent stones and/or cholangitis after treatment." 11/01/2013 - "Hepatobiliary complications, such as stone recurrence, recurrent cholangitis, liver abscess, secondary biliary cirrhosis, and cholangiocarcinoma may occur after treatment for hepatolithiasis. "
5.	Hormone Replacement Therapy (Therapy, Hormone Replacement) 07/01/2004 - "An open, randomized, controlled study of transdermal hormone replacement therapy on the rate of bone loss in primary biliary cirrhosis." 03/01/2004 - "Transdermal hormone replacement therapy improves vertebral bone density in primary biliary cirrhosis: results of a 1-year controlled trial." 03/01/2004 - "Retrospective studies have suggested that hormone replacement therapy may reduce the rate of bone loss in primary biliary cirrhosis, but no controlled data are available. " 03/01/2004 - "Compared with calcium and vitamin D alone, supplemental treatment with transdermal hormone replacement therapy for 1 year improved the vertebral bone density and urinary markers of bone turnover in post-menopausal women with primary biliary cirrhosis." 03/01/2004 - "Forty-two post-menopausal women with primary biliary cirrhosis were treated with calcium and vitamin D, either alone (n = 21) or together with transdermal hormone replacement therapy (n = 21). "