Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autoimmune hepatitis is characterized by hypergammaglobulinemia, female predominance, autoantibodies and a good response to immunosuppression, and is based on specific autoantibodies and clinical characteristics. Several subgroups may be distinguished. As in most autoimmune diseases, the etiology is unknown. The association of autoimmune hepatitis with a viral etiology is most prominent in autoimmune hepatitis type 2 which is characterized by liver/kidney microsomal (LKM-1) autoantibodies against cytochrome P-450 II D6. Depending on the geographical origin of the patients, a specific proportion of patients with autoimmune hepatitis type 2 is associated with hepatitis C virus infection. These HCV RNA-positive patients are older, female predominance is not profound, and response to immunosuppression is generally low compared to the HCV-negative patients with autoimmune hepatitis type 2. The genetic background is unclear. HCV sequence analysis revealed that HCV genotype II is prominent in HCV-positive autoimmune hepatitis type 2. HCV mutants with deletions in the HCV envelope region were identified. The relevance of these HCV mutants for the induction of autoimmunity needs to be characterized further. The HCV-negative population of patients with autoimmune hepatitis type 2 seems to have a relation with herpes simplex virus (HSV-1) infection since the B-cell epitope of cytochrome P-450 II D6, the major LKM-1 antigen, shares sequence homology with the IE-175 protein of HSV-1. In the HCV-negative population of autoimmune hepatitis type 2, HLA-DR3 and C4-AQ0 alleles are significantly increased.
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PMID:Viruses and autoimmune liver disease. 840 37

An etiopathological link between hepatitis virus infection and autoimmune liver disease, in particular autoimmune hepatitis has been suggested. In some patients features of both viral and autoimmune disease are present. We have studied 352 patients with autoimmune liver disease and 507 patients with viral hepatitis for diagnostic characteristics as well as for evidence of an etiological connection. 38 of the 201 patients with hepatitis C (19%) and 42 of the 306 patients with hepatitis B (14%) had significant titres of autoantibodies (ANA, SMA or LKM). SLA autoantibodies were found exclusively in patients with autoimmune liver disease. LKM auto-antibody was found in only one of the 201 HCV patients. Evidence of past or present hepatitis B virus and past hepatitis A virus infection was most common in the hepatitis C virus patients and least common in autoimmune hepatitis. 28 of the 352 patients with autoimmune liver diseases tested positive in the second generation anti-HCV ELISA, but only five patients (two with autoimmune hepatitis, one with primary sclerosing cholangitis and two with primary biliary cirrhosis) were positive in confirmatory anti-HCV assays, and only in these could HCV-RNA be isolated. Autoimmune hepatitis patients had significantly higher transaminase, GLDH and IgG levels. HLA-B8, HLA-DR3 and HLA-DR4 were significantly more common in autoimmune hepatitis. Distinction between autoimmune liver disease and viral hepatitis C could be made reliably on clinical and laboratory grounds. Our data show that a link between hepatitis A, B, or C virus infection and autoimmune liver diseases is highly unlikely.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Relation between autoimmune liver diseases and viral hepatitis: clinical and serological characteristics in 859 patients. 852 56

Autoimmune hepatitis is an inflammatory disease of the liver of unknown etiology that progresses toward cirrhosis and liver failure and is generally responsive to immunosuppressive treatment. The presence of anti-smooth muscle antibodies with anti-actin specificity and of anti-liver kidney microsomal antibodies defines two distinct subgroups of the disease. An autoantibody against liver cytosolic antigens has recently been described. Management of autoimmune hepatitis relies on immunosuppressive therapy with steroids alone or combined with azathioprine. When the disease is poorly controlled, despite good patient compliance to therapy, cyclosporin should be recommended. Progressive liver disease in chronic hepatitis B in adults has been associated with the presence of precore mutants of hepatitis B virus. In children, the presence of precore mutants seems not to affect the rate of seroconversion to anti-hepatitis B e antigen. However, high viremic levels of precore mutants are associated with persistent viral replication and liver disease. Interferon alfa seems to be less effective in children than in adults in the treatment of chronic hepatitis B; however, it hastens the seroconversion rate to anti-hepatitis B e antigen, accelerating the spontaneous clearance of the virus in children with already low levels of viral replication. Blood transfusions, especially those received in the perinatal period, are the single most important source of infection with hepatitis C in childhood. HIV coinfection is a major risk factor for vertical transmission of hepatitis C virus in pregnant women. Chronic hepatitis C in children is usually an asymptomatic disease associated with mild to moderate fluctuation of aminotransferase activities and histologic features of mildly active hepatitis. Severe active hepatitis and cirrhosis are infrequent during childhood and adolescence. Interferon may have a place in the treatment of chronic hepatitis C in children.
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PMID:Chronic hepatitis in children. 854 54

We have reclassified 110 patients with autoantibody-positive cryptogenic chronic hepatitis according tot he aggregate scoring system proposed by the International Autoimmune Hepatitis Group for signs of hepatitis C virus (HCV) infection and the newly proposed terminology of "unclassified" chronic hepatitis. Anti-HCV and HCV viremia were assessed by second-generation assays and reverse transcription-polymerase chain reaction. Immunomorphological and immunochemical characterizations of antinuclear, smooth, muscle, liver-kidney microsomal type 1, and liver cytosol type 1 autoantibodies were also performed. All 45 anti-HCV negative patients fulfilled the score criteria for the diagnosis of "definite" or "probable" autoimmune hepatitis (AIH). Eight anti-HCV-positive cases reached the score of "probable" AIH, whereas the remaining 57 cases were diagnosed as unclassified chronic hepatitis. The scoring system allows the correct identification of all autoimmune cases without HCV infection. Autoimmune hepatitis runs a more severe disease course than unclassified chronic hepatitis, whose clinical and histological features are similar to those of autoantibody-negative chronic hepatitis C.
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PMID:Impact of international autoimmune hepatitis group scoring system in definition of autoimmune hepatitis. An Italian experience. 856 51

The morphology of autoimmune hepatitis is characterized by portal-periportal predilection of necroinflammatory lesions. In comparison to the viral type of hepatitis severe piece-meal-necroses, the collapse of periportal parenchyma, and to a higher degree acinar transformation of hepatocytes are more prominent. The autoimmune hepatitis may start with acute disease displaying unusual clinical und histopathologic features. The postinfantile giant cell hepatitis seems to constitute a variant of autoimmune hepatitis. Autoimmune hepatitis has been reproduced in animal models and it could be demonstrated in rabbits that humoral immunity plays a role in tissue damage. The importance of cellular mechanisms could be analyzed in syngenic mice showing that the CD4-positive lymphocytes play a pivotal role. The most promising candidate antigen seems to be the asialoglycoprotein-receptors including the liver specific protein (LSP). By immunohistologic analysis dense deposits of IgG could be demonstrated in sinusoids and on the membranes of hepatocytes. In accordance with in vitro data the determination of CD4 positive lymphocytes in the tissue was found to play a decisive role in cellular immune reaction. The HSP65 molecule seems to evoke mechanisms that have been shown to play a pathogenetic role in experimental arthritis.
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PMID:[Autoimmune hepatitis]. 860 Jun 83

Chronic inflammatory liver diseases can be induced by virus infections, toxic-metabolic factors and/or autoimmune mechanisms. This overview deals with the immunopathogenesis of chronic hepatitis B and C and autoimmune hepatitis (AIH). 1. Chronic hepatitis B: The immune response to HBV-antigens is responsible both for viral clearance and disease pathogenesis during HBV-infection. The humoral immune response to HBsAg contributes to the clearance of circulating virus particles, the cell mediated immune response to HBsAg, HBcAg and polymerase antigen eliminates infected cells. The class I- and class II restricted T-cell-responses to HBV is strong, polyclonal and multispecific in acute HB with successful clearance of the virus, but weak or incomplete in chronic HB with viral persistence. In addition to ineffective immune response host and viral factors as well as abnormalities in virus-host interactions may be the main reasons for the maintenance of HBV-carrier status. 2. Chronic hepatitis C develop in more than 60% of infected patients. There is increasing evidence that the immune response to HCV-epitopes plays an important role in the course and the pathogenesis of the disease. It has been shown that CD4+ and CD8+ T-cells recognize viral peptides in the presence of class I and II molecules. The fine specificity and functional significance of liver infiltrating and peripheral blood T-cells demonstrate HCV specific immunodominant epitopes targeted by class Ii restricted CD4+ cells in patients with chronic HCV infection. The T-cell response correlates with disease activity. The cytokine release of T-cells resemble a TH1-like profile. Studies of the humoral immune response to HCV show a correlation between IgM-anti-HCV and disease activity. In vitro and in vivo anti-HCV secretion by PBMC is due to persistent antigenic stimulation of B-cells by ongoing production of viral antigens and reflects HCV replication in PBMC. Of special interest are several immune mediated disease and immune abnormalities in chronic hepatitis C. 3. Autoimmune hepatitis (AIH) is a distinct group of acute and chronic necro-inflammatory disorders of unknown etiology characterized by immunological and autoimmunological features including the presence of autoantibodies but without an antecedent of viral infections. Marker autoantibodies define 3 subtypes: Type I (ANA/SMA), Type II (LKM1-AB), Type II (SLA-AB). AIH is associated with a distinct genetic background (HLA A1, B8, DR3 or DR4). Several studies clearly demonstrate that liver cell damage in AIH is mediated by autoimmune reactions against normal constituents of hepatocytes. Although the precise mechanisms are not yet fully understood, there is now considerable evidence that autoantigens of the hepatocellular membrane in particular the ASGPR are important targets of liver damaging autoreactions in AIH. Cellular and humoral immune reactions against the human ASGPR correlate with disease activity and usually disappear under immunosuppressive therapy.
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PMID:[Immunopathology of chronic liver diseases]. 860 Jun 84

To assess the validity of a scoring system developed by the International Autoimmune Hepatitis Group for the definite diagnosis of autoimmune hepatitis, 119 patients with autoimmune hepatitis by standard clinical criteria and 131 patients with other chronic liver diseases were evaluated. Each patient was graded on 35 items in 13 clinical categories. Ninety-seven patients diagnosed as having autoimmune hepatitis by conventional criteria (82%) had a definite diagnosis by the scoring system and 22 patients (18%) had a probable diagnosis. Of these patients, those with definite diagnoses had significant clinical differences from those with probable diagnoses. Only two patients with other chronic liver disease (2%) had scores sufficient for a definite diagnosis of autoimmune hepatitis. Probable diagnoses, however, were common in other conditions (33%). Failure to adequately downgrade for cholestatic features contributed to these uncertain diagnoses. Scoring for treatment response downgraded the diagnosis in 17 of 93 patients with a definite diagnosis before therapy (18%) and upgraded the diagnosis in 6 of 14 others (43%). We conclude that the scoring system is specific for the definite diagnosis of autoimmune hepatitis, and it complements standard clinical criteria by establishing the strength of the diagnosis and defining distinctive subgroups within the diagnostic category. Refinements are necessary to reduce the frequency of probable diagnoses. Diagnoses at presentation can be commonly modified by scoring treatment response.
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PMID:Validation of scoring system for diagnosis of autoimmune hepatitis. 860 74

Overlapping features between primary sclerosing cholangitis (PSC and autoimmune hepatitis (AIH) have previously been noted. To assess systematically similarities between these disorders, we have evaluated 114 PSC patients (36 women; 78 men), all confirmed by endoscopic retrograde cholangiography (ERC), according to a scoring system proposed by The International Autoimmune Hepatitis Group for the diagnosis of AIH. The scoring system attributes positive or negative scores to the parameters sex, ratio of elevation of serum levels of alkaline phosphatase (ALP) vs. aminotransferase, serum levels of immunoglobulins and autoantibodies, viral markers, history of drug and alcohol intake, genetic factors, liver histology, and response to therapy. Two of the PSC patients (2%) obtained scores above 15 before treatment, satisfying the diagnostic criterion of "definite" AIH. Thirty-eight patients (33%) scored between 10 and 15 points and could be classified as "probable" AIH. The serum level of immunoglobulin G (IgG) was elevated in 68 patients (61% of 111 cases tested), and positive titers of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) were detected in 24 patients (22% of 111 cases tested). Thirty-five of the PSC patients (33% of 105 evaluable biopsy specimens) obtained positive scores for histological features similar to those of AIH, but the total score for histology was in the negative range in 72 patients (69%) because of the presence of biliary changes. The frequent finding of high scores in PSC patients underlines the similarities PSC may have with AIH. A modification of the scoring system, in particular by increasing the negative score for histological biliary changes, would improve its potential to discriminate between AIH and PSC.
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PMID:Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis. 867 53

Autoimmune hepatitis (AIH), a liver inflammatory condition, is characterized by hypergammaglobulinemia, hypertransaminasemia, presence of autoantibody and periportal hepatitis revealed by histology. Usually it comes with acute onset and with bad prognosis. We describe recent updated literature for clinical manifestation and pathogenesis, principally referring to adult disease. We described the data obtained from a multicenter investigation (10 Italian center) included in the Gastroenterology and Hepatology Italian Society. The investigation collected 42 AIH cases in 18 years, this confirms that the disease is rare for pediatric age. There is a prevalence of females on males but not as important as in adults (F/M:3/1). The average age at diagnosis was 7 + 4 years old. The diagnosis was always made based on observed symptoms excluding 2 cases with silent hypertransaminasemia. The most frequently observed autoantibody was SMA. In two cases, diagnosis was done without finding commonly investigated autoantibody. Prognosis is severe, in one case the hepatic transplantation was successful.
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PMID:[Autoimmune hepatitis: pediatric aspects]. 876 73

We describe a hepatitis B virus carrier who satisfied the criteria of autoimmune hepatitis proposed by the International Autoimmune Hepatitis Group. A 43-year-old Japanese female showed human leukocyte antigen typing including DR4 in addition to hypergammaglobulinemia, presence of autoantibodies, and liver histology suggestive of autoimmune hepatitis. Moreover, the predominant presence of hepatitis B core antigen in nuclei rather than in cytoplasm of hepatocytes suggested less of a possibility of liver cell damage related to hepatitis B virus infection. She completely responded to immunosuppressive therapy and no clinical or biochemical relapse has been recognized to date.
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PMID:Hepatitis B virus carrier status linked to autoimmune hepatitis. 883 98


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