Positive markers in AMA-negative PBC

Vergani, D

doi:10.1016/s0002-9270(02)05938-5

Cited by 15 publications

(15 citation statements)

References 13 publications

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“…This includes the presence of autoreactive T and B cells against specific antigens in the peripheral blood and the inflamed liver tissue of PBC patients and high-titre serum autoantibodies characteristic of the disease [69, 75, 105–114]. There is also evidence in support of infectious agents and xenobiotics mimicking the major autoantigen in PBC, PDC-E2, can induce loss of immunological tolerance and biliary epithelial-specific pathology, leading to the destruction of the bile ducts [22, 115–117].…”

Section: Ebv and Pbcmentioning

confidence: 99%

Epstein-Barr Virus as a Trigger of Autoimmune Liver Diseases

Rigopoulou

Smyk

Matthews

et al. 2012

Advances in Virology

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The pathogenesis of autoimmune diseases includes a combination of genetic factors and environmental exposures including infectious agents. Infectious triggers are commonly indicated as being involved in the induction of autoimmune disease, with Epstein-Barr virus (EBV) being implicated in several autoimmune disorders. EBV is appealing in the pathogenesis of autoimmune disease, due to its high prevalence worldwide, its persistency throughout life in the host's B lymphocytes, and its ability to alter the host's immune response and to inhibit apoptosis. However, the evidence in support of EBV in the pathogenesis varies among diseases. Autoimmune liver diseases (AiLDs), including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), have a potential causative link with EBV. The data surrounding EBV and AiLD are scarce. The lack of evidence surrounding EBV in AiLD may also be reflective of the rarity of these conditions. EBV infection has also been linked to other autoimmune conditions, which are often found to be concomitant with AiLD. This paper will critically examine the literature surrounding the link between EBV infection and AiLD development. The current evidence is far from being conclusive of the theory of a link between EBV and AiLD.

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Section: Ebv and Pbcmentioning

confidence: 99%

Epstein-Barr Virus as a Trigger of Autoimmune Liver Diseases

Rigopoulou

Smyk

Matthews

et al. 2012

Advances in Virology

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“…Hence, most investigators have demonstrated certain immunologic features that distinguish AMA-positive and AMA-negative cases, particularly because there does not seem to be consistent clinical, biochemical, or histologic differences. Most observe a lower serum IgM concentration in patients who are AMA negative, with an accompanying higher IgG level [10,12] and a higher frequency of positive immunofluorescence for antinuclear antibodies (ANA) and smooth muscle antibodies [36][37][38][39]. This is part of the basis for the variety of labels previously used in describing the condition.…”

Section: Disease Manifestationsmentioning

confidence: 99%

Antimitochondrial Antibody-Negative Primary Biliary Cirrhosis

Hirschfield

Heathcote

2008

Clinics in Liver Disease

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“…The presence of ANA at diagnosis seems to be able to identify individuals who will develop advanced disease faster than those seronegative for these autoantibodies [37]. It should be noted that patients may initially be found to be seronegative for AMA or disease-specific ANA with conventional techniques such as that of indirect immunofluorescence (IFL) [20, 38–40]. More sensitive tests using as antigenic source hybrids containing the major mitochondrial antigens have led to the appreciation that “true” AMA-seronegative cases may exist but are fewer than those considered in the past when conventional AMA testing was based on IFL and enzyme immunoassays based on the M2 antigen [20, 38–40].…”

Section: Introductionmentioning

confidence: 99%

Sex Differences Associated with Primary Biliary Cirrhosis

Smyk

Rigopoulou

Pares

et al. 2012

Clinical and Developmental Immunology

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Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7–11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.

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Positive markers in AMA-negative PBC

Cited by 15 publications

References 13 publications

Epstein-Barr Virus as a Trigger of Autoimmune Liver Diseases

Epstein-Barr Virus as a Trigger of Autoimmune Liver Diseases

Antimitochondrial Antibody-Negative Primary Biliary Cirrhosis

Sex Differences Associated with Primary Biliary Cirrhosis

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