Search results for "PRIMARY BILIARY CIRRHOSIS"
showing 10 items of 61 documents
Clonal analysis of human T lymphocytes infiltrating the liver in chronic active hepatitis B and primary biliary cirrhosis
1988
Human T lymphocytes infiltrating the liver in chronic active hepatitis B (CAH-B) and primary biliary cirrhosis were isolated from liver biopsy cores, cloned by limiting dilution technique and expanded in vitro. Phenotypic and functional analysis demonstrates that this tissue infiltrate represents a heterogeneous cell population. However, when compared to peripheral blood lymphocytes of the same patients, a marked enrichment for T8+ cytotoxic T cells was found to exist at a local site in both types of chronic liver disease. These data provide support for the notion that liver cell injury in CAH-B and PBC may be mediated by a common immunologic mechanism likely executed by cells of the T line…
Identification of target antigen for SLA/LP autoantibodies in autoimmune hepatitis.
2000
Summary Background Autoantibodies are a hallmark of autoimmune hepatitis, but most are not disease specific. Autoantibodies to soluble liver antigen (SLA) and to liver and pancreas antigen (LP) have been described as disease specific, occurring in about 30% of all patients with autoimmune hepatitis, but no standardised assays are available. Methods We tested 2000 serum samples from patients with various liver diseases and controls for SLA autoantibodies by inhibition ELISA. Serum samples positive for SLA antibodies were used for immunoscreening of cDNA expression libraries. Identified clones were tested against a panel of serum samples positive for SLA and LP autoantibodies and control seru…
Immune Reactions in Liver Diseases
1983
Chronic hepatitis is a heterogeneous syndrome with different underlying etiologies. Viruses (hepatitis B virus, non-A, non-B viruses), drugs and even alcohol are regarded as etiological agents. A further subgroup is autoimmune-type chronic hepatitis in which an immunogenetic predisposition is of major importance. Primary biliary cirrhosis has to be separated from these subgroups of chronic hepatitis.
Evidence for spontaneous immunosuppression in autoimmune hepatitis
1995
Autoimmune hepatitis (AIH) runs a variable clinical course. Slow disease progression or even spontaneous remissions can be observed and suggest that the autoimmune process can, at least to a certain extent, be controlled by regulatory elements of the patient's own immune system. In experimental autoimmune hepatitis (EAH) spontaneous recovery is regularly observed and associated with antigen-specific and antigen-nonspecific suppression. The aim of the current study was to search for similar immunoregulatory phenomena in patients with AIH. We examined T-cell reactivity to soluble human liver antigens in 11 patients with active autoimmune hepatitis and 30 patients with other liver diseases (ch…
Characterization of Anti Liver Kidney Microsomal Antibody Associated with Chronic HDV Infection by Immunoblotting
1989
Cytoplasmic antibodies directed against antigen(s) present in the membranes of the endoplasmic reticulum have been reported in a proportion of patients with chronic HBV-HDV infection (Crivelli et al.,1981). While the immunofluorescence (IFL) pattern associated with LKMδ antibody is quite similar to that of the liver kidney microsomal antibody (LKM) described by Rizzetto et al. (1973) and associated with an autoimmune type of chronic active hepatitis (Thomas, 1980), the target antigen(s) was supposed to be different on the basis of blocking experiments. The target antigen of LKM antibody has been recently partially identified as a 50kD protein located in the membranes of the smooth endoplasm…
Autoimmune Lebererkrankungen: Diagnostik und Therapie
2004
Autoimmune Hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and overlap syndromes of these three disease entities are regarded as autoimmune liver diseases. These conditions are important differential diagnoses of elevated liver function tests as about 10 % of liver transplantations in Europe and North America are for these indications. The diagnosis is often difficult but can be facilitated by sequential measurement of relevant autoantibodies, exclusion of other liver disease, ultrasound, ERCP and liver histology. In AIH immunosuppressive therapy has been shown to prevent or stop the development of cirrhosis and improve the prognosis of the patients de…
Heterozygosity for the hemochromatosis gene in liver diseases - prevalence and effects on liver histology
2000
:Background/Aims: The effect of heterozygosity for the C282Y mutation in the HFE hemochromatosis gene on iron accumulation and disease progression in liver disease patients is unclear. Methods: We investigated the prevalence of this mutation in 531 patients and 205 healthy controls. In addition, we assessed the influence of the mutation on liver histology in 34 C282Y heterozygous and 124 age-, sex- and disease-matched controls without the mutation using the modified HAI and Chevallier score. Results: The highest prevalence of the C282Y mutation was observed in patients with autoimmune hepatitis (17.2%, p<0.01) compared to 6.4% in healthy controls. Heterozygotes with hepatitis C and B virus …
Multicentre Survey of the Prevalence of Intrahepatic Cholestasis in 2520 Consecutive Patients with Newly Diagnosed Chronic Liver Disease
1992
In order to establish the prevalence of intrahepatic cholestasis (IHC) in chronic liver disease, we retrospectively evaluated the frequency of increased serum total bilirubin (STB), serum conjugated bilirubin (SCB) and serum alkaline phosphatase (SAP) levels in a cohort of 2520 patients with newly diagnosed chronic liver disease presenting over 2 years. 882 patients (35%) [mean age 52.2 years] had IHC involving abnormal levels of STB (mean 89.8 μmol/L), SCB (mean 52.5 μmol/L), and SAP (mean 5.7 μkat/L) together with a negative ultrasonic scan. Demographic data were not predictive of IHC. IHC was more frequent in patients with cirrhosis (43%), primary biliary cirrhosis (54%), and primary scl…
Pyruvate dehydrogenase specific T cells in primary biliary cirrhosis show restricted antigen recognition sites
2002
: Background/Aims: The aim was to characterise the antigen recognition sites of the variable T cell receptor α-chain (TCRAV) and β-chain (TCRBV) of T cells specific to the pyruvate dehydrogenase (PDC) in primary biliary cirrhosis. Methods: In 21 PDC-specific T cell clones isolated from five patients we analysed TCRAV and TCRBV usage by RT-PCR and sequenced the CDR3 regions. Results: Preferential expression of the TCR elements BV6 (6 clones), BV12 (4 clones) and BV1 (3 clones), and frequent usage of the joining elements JB2.3 and JB2.1 were seen. Analysis of the α chain revealed rearrangement of AV2 in 7 clones (35%) and AV7 in 3 clones, however, distribution of the joining elements was het…
Antimitochondrial antibody -M2 positive autoimmune hepatitis during standard of care for chronic hepatitis C
2012
The current standard of care (SoC) for chronic hepatitis C, i.e. the combination of a pegylated-interferon (PEG-IFN) with ribavirin (RBV), may activate underlying autoimmune conditions. Particularly, interferon (IFN) has been known to induce or exacerbate autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) in hepatitis C virus patients. We describe a severe, acute-onset antimitochondrial antibody (AMA)-M2 positive AIH appearing during the last weeks of SoC in a woman with chronic hepatitis C and no previous history of autoimmunity, and resolving on protracted steroids. In this context, the relevance of the characterization of the immunoglobulin isotype of portal plasma cells for …