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 cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.

Also Known As:

Networked: 4195 relevant articles (190 outcomes, 424 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Gershwin, M Eric: 135 articles (10/2015 - 01/2002)
2.	Invernizzi, Pietro: 53 articles (02/2016 - 07/2003)
3.	Coppel, Ross L: 50 articles (11/2014 - 01/2002)
4.	Ansari, Aftab A: 49 articles (11/2014 - 01/2002)
5.	Selmi, Carlo: 47 articles (08/2015 - 01/2002)
6.	Lindor, Keith D: 43 articles (01/2016 - 01/2002)
7.	Nakanuma, Yasuni: 43 articles (10/2015 - 01/2002)
8.	Gershwin, M E: 31 articles (06/2015 - 01/2000)
9.	Podda, Mauro: 31 articles (04/2014 - 01/2002)
10.	Ishibashi, Hiromi: 30 articles (02/2014 - 04/2002)

Drugs and Biologics

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

1.	Ursodeoxycholic Acid (Urso)FDA LinkGeneric 11/01/2000 - "Ursodeoxycholic acid (UDCA) is a safe and effective medical therapy for most patients with primary biliary cirrhosis (PBC), but some patients show an incomplete response. " 02/01/2000 - "Ursodeoxycholic acid (UDCA) is a safe and effective medical therapy for most patients with primary biliary cirrhosis (PBC). " 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. " 05/01/2005 - "This case report indicates a serological remission and a marked histological improvement in a female patient with an early stage of primary biliary cirrhosis (stage I) during therapy under ursodeoxycholic acid. " 02/01/2005 - "Despite evidence for therapeutic efficacy with ursodeoxycholic acid (UDCA) in primary biliary cirrhosis (PBC), only 30-50% of patients achieve complete biochemical remission within 1 year of therapy. "
2.	Primary sclerosing cholangitisIBA 01/01/1992 - "UDCA seems safe and effective in the early stages of primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and possibly in other severe cholestatic syndromes of infancy. " 06/28/1990 - "Since the association of primary sclerosing cholangitis with HLA-DRw52a appears to be total, HLA typing should be helpful in differentiating this disease from primary biliary cirrhosis." 05/01/2013 - "Recent human and animal studies using bile acid analogues suggest a therapeutic potential for TGR5 in primary biliary cirrhosis but not in primary sclerosing cholangitis. " 05/01/2012 - "Studies on the epidemiology of primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) show variable outcome. " 04/01/2012 - "This study compares serum bile acid levels in patients with primary biliary cirrhosis and primary sclerosing cholangitis and from age and sex-matched non cholestatic donors. "
3.	ColchicineFDA 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. " 12/01/1991 - "We followed up a group of patients with primary biliary cirrhosis who participated in a 4-yr prospective, double-blind controlled trial of colchicine therapy for 4 additional years. " 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."
4.	BezafibrateIBA 02/01/2005 - "The therapeutic efficacy of bezafibrate, a hypolipidemic drug, has been shown in patients with primary biliary cirrhosis (PBC) in some pilot studies; however, little is known regarding the mechanism of action of bezafibrate in PBC. " 02/01/2005 - "Therapeutic efficacy of decreased nitrite production by bezafibrate in patients with primary biliary cirrhosis." 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." 01/01/2012 - "We need more randomised clinical trials on the effects of bezafibrate on primary biliary cirrhosis with low risks of systematic errors and random errors."
5.	BilirubinIBA 08/01/2012 - "[A clinical study of bilirubin rise in short-term in patients with primary biliary cirrhosis]." 09/01/2015 - "New simple prognostic score for primary biliary cirrhosis: Albumin-bilirubin score." 02/10/2015 - "We apply the procedure to estimate survival time-dependent cut-point on the prognostic biomarker of serum bilirubin among 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. "
6.	Methotrexate (Mexate)FDA LinkGeneric 11/01/1991 - "The data suggest that methotrexate may be effective treatment for precirrhotic primary biliary cirrhosis. " 02/01/1996 - "To assess the efficacy of low doses of oral methotrexate as therapy for primary biliary cirrhosis. " 01/01/2005 - "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."
7.	Immunoglobulin G (IgG)IBA 05/01/2014 - "We measured total IgG and IgG subclasses in serum samples of IAC (n = 73) and PSC (n = 310) patients, as well as in serum samples of disease controls (primary biliary cirrhosis; n = 22). " 09/01/2009 - "Serum antimitochondrial antibody (AMA) of the IgG2 and IgG3 subclasses has been reported to be predominant in patients with primary biliary cirrhosis from developed countries. " 03/01/2009 - "Multiple nuclear dots and rim-like/membranous IgG isotypes in primary biliary cirrhosis." 09/01/2006 - "We measured serum IgG4 in 127 patients with PSC and 87 patients with primary biliary cirrhosis, as disease controls. " 09/01/1995 - "Acquired total immunoglobulin G deficiency in a patient with primary biliary cirrhosis."
8.	AntibodiesIBA 11/01/2014 - "The aim of this study was to evaluate the diagnostic value of anti-mitochondrial antibodies (AMAs) and/or the M2 subtype (AMA-M2) in patients with primary biliary cirrhosis (PBC). " 01/01/2012 - "In a recent study we showed that in patients with primary biliary cirrhosis (PBC) being positive or negative for anti-M2 antibodies reacting with the 2-oxoacid-dehydrogenase complex (ODC) also antibodies to the beta- and gamma-subunits of F1F0-ATPase (anti-β, anti-γ) occur. " 07/01/2008 - "The aim of this study was to evaluate, retrospectively, the frequency of anti-Saccharomyces cerevisiae antibodies (ASCA) in patients with primary biliary cirrhosis (PBC). " 01/01/2005 - "Double-blind, single-blind, or unblinded randomised clinical trials evaluating any preparation of glucocorticosteroids versus placebo or no intervention in patients with primary biliary cirrhosis diagnosed by abnormal liver function tests and either anti-mitochondrial antibodies or histology were included. " 12/01/1997 - "Clinicopathological study of primary biliary cirrhosis negative for antimitochondrial antibodies."
9.	Immunoglobulin M (IgM)IBA 01/01/1990 - "In conclusion this study suggests that during the enhanced IgM synthesis observed in primary biliary cirrhosis a defect occurs in the assembly of the IgM pentamer with release of monomeric and oligomeric IgM into the circulation. " 08/01/1985 - "To evaluate the mechanisms underlying the increase in serum IgM in primary biliary cirrhosis (PBC) studies were designed to examine IgM production in vitro and to assess the relative contribution of intrinsic B cell activity and immunoregulatory T cell balance to IgM synthesis. " 08/01/1985 - "In vitro studies on the mechanism of increased serum IgM levels in primary biliary cirrhosis." 01/01/1981 - "Immunofluorescence studies on skin biopsies from 14 patients with primary biliary cirrhosis (PBC) showed granular papillary deposition of IgM in all. " 02/01/2007 - "We previously reported that peripheral blood mononuclear cells (PBMCs) from patients with primary biliary cirrhosis (PBC) produce significantly higher levels of polyclonal IgM than controls after exposure to CpG. "
10.	Cyclosporine (Ciclosporin)FDA LinkGeneric 11/01/1988 - "The results of this study indicate that in symptomatic primary biliary cirrhosis, cyclosporin A administration is associated with a significant improvement in cholestatic liver enzyme abnormalities that persists for the duration of therapy. " 11/01/1988 - "The purpose of this pilot study was to determine whether daily administration of cyclosporin A to symptomatic patients with primary biliary cirrhosis for 1 yr would lead to a significant and sustained improvement in liver enzyme abnormalities. " 01/01/2007 - "Cyclosporin A has been used for patients with primary biliary cirrhosis, but the therapeutic responses in randomised clinical trials have been heterogeneous. " 05/17/1990 - "A controlled trial of cyclosporine in the treatment of primary biliary cirrhosis." 10/01/1984 - "Pharmacokinetic study of cyclosporin A (Sandimmun) in patients with primary biliary cirrhosis."

Therapies and Procedures

1.	Liver Transplantation 08/01/2003 - "Generally, the most effective treatment for advanced primary biliary cirrhosis (PBC) is liver transplantation, but adjunct therapies are needed. " 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). "
2.	Transplants (Transplant) 10/17/2015 - "Several randomised placebo-controlled studies have shown that UDCA improves transplant-free survival in primary biliary cirrhosis. " 06/01/2010 - "This study of 100 women with primary biliary cirrhosis who were either waiting for or had already had a liver transplant focused on both changes in uncertainty with transplant and the correlates of uncertainty both pre- and post-transplant. " 07/01/2001 - "Many clinical trials of UDCA in a variety of cholestatic disorders established biochemical and clinical improvements, and most importantly showed a significant prolongation of transplant-free survival after four years of treatment with UDCA in patients with primary biliary cirrhosis [5]. " 08/01/1989 - "Histologic studies on the hepatic lesions induced by graft-versus-host reaction in MHC class II disparate hosts compared with primary biliary cirrhosis." 01/01/2015 - "Among immune-mediated disorders, primary biliary cirrhosis recurrence affects 30 - 50% of transplant patients, albeit with mild consequences. "
3.	Transplantation (Transplant Recipients) 09/27/2001 - "The prognosis of patients with primary biliary cirrhosis has improved since the introduction of transplantation. " 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. " 03/01/2013 - "The indication for transplantation was secondary biliary cirrhosis. "
4.	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). "
5.	Estrogen Replacement Therapy 04/01/2003 - "Estrogen replacement therapy in postmenopausal patients with primary biliary cirrhosis is safe and may be effective in decreasing the rate of bone loss." 04/01/2003 - "The aim of the present study was to determine the safety as well as the efficacy of estrogen replacement therapy with respect to bone mass in postmenopausal women with primary biliary cirrhosis. " 01/01/1994 - "This study should serve as an impetus for a controlled trial of estrogen replacement in postmenopausal patients with primary biliary cirrhosis." 04/01/2003 - "Bone mineral density of the lumbar spine (measured using dual energy x-ray absorptiometry) of 46 unselected postmenopausal women with primary biliary cirrhosis receiving estrogen replacement therapy was compared with 46 age-matched women with primary biliary cirrhosis not receiving estrogen replacement therapy, and with the expected normal bone mineral density adjusted for age and ethnic group. "