Autoimmune hepatitis

Mieli‐Vergani, Giorgina; Vergani, Diego; Czaja, Albert J.; Manns, Michael P.; Krawitt, Edward L.; Vierling, John M.; Lohse, Ansgar W.; Montano‐Loza, Aldo J.

doi:10.1038/nrdp.2018.17

Cited by 346 publications

(416 citation statements)

References 220 publications

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“…Histologic studies across all 3 diseases with phenotypic analysis of infiltrating lymphocytes support this [5] . T-Cells and Immune Tolerance T-lymphocytes have been shown to predominate over other cell types in areas of interface hepatitis seen in AIH [21] . T-lymphocytes develop in the thymus where they interact with thymic epithelial cells (TECs), which express self-antigens.…”

Section: Immunologymentioning

confidence: 99%

The Pathogenesis of Autoimmune Liver Disease

Arndtz

Hirschfield

2016

Dig Dis

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Background: Autoimmune liver disease (AILD) encompasses 3 main distinct clinical diseases: autoimmune hepatitis, primary biliary cholangitis (formally known as cirrhosis, PBC) and primary sclerosing cholangitis (PSC). These conditions are an important, yet under-appreciated cause of patient morbidity and mortality with ongoing unmet needs for further research and clinical advances. Key Messages: There is observational evidence for genetic predisposition, with all 3 conditions being more common in first degree relatives. AILD is associated with the presence of auto-antibodies and higher risks of other non-hepatic auto-immune conditions. Genetic risk association studies have identified HLA and non-HLA risk loci for the development of disease, with some HLA loci providing prognostic information. This re-enforces the concept that genetic predisposition to autoimmunity is important, likely in the context of environmental exposures. Such environmental triggers are unclear but relevant risks include smoking, drug and xenobiotic exposure as well as the complexities of the microbiome. There is evidence for a loss of immune tolerance to self-antigens playing a part in the development of these conditions. In particular the IL-2 and IL-12 regulatory pathways have been implicated in pre-disposing to an unopposed inflammatory response within the liver. Main immunological themes revolve around loss of immune tolerance leading to T-cell mediated injury, imbalance in the regulation of immune cells and defective immune response to foreign antigens. For PBC and PSC, there is then the added complexity of the consequences of cholestasis on hepato-biliary injury, immune regulation and liver fibrosis. Conclusions: Whilst specific disease causes and triggers are still lacking, AILD arises on the background of collective genetic and environmental risk, leading to chronic and abnormal hepato-biliary immune responses. Effective and more rational therapy will ultimately be developed when the multiple pathways to liver injury are better understood.

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Section: Immunologymentioning

confidence: 99%

The Pathogenesis of Autoimmune Liver Disease

Arndtz

Hirschfield

2016

Dig Dis

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“…[5,14] Autoimmune Hepatitis: (AIH)has broad clinical spectrum including asymptomatic individuals with abnormal laboratory results, clinical symptoms similar to those of acute viral hepatitis, and hepatic insufficiency or even cirrhosis . [15,14 ] The cases were diagnosed based on characteristic histologic features like interface hepatitis and dense infiltration of portal tracts by mononuclear cells and predominance of plasma cells. In a study by Jimenez-Riviera et al, of 200 cases, the median age range was 12 years for type 1 AIH and 10 years for Type 2 AIH.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Liver Biopsy: A clinicopathologic study

Sathe

Mahale

2017

APALM

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Extrahepatic Biliary Atresia: (Fig 1 a, b) -A total of 26 cases of EHBA were found in our study. Among these, 25 were infants. The commonest symptom was icterus followed by clay colored stools. Liver function test results were available in 17 cases. Liver enzymes were elevated in the following range -SGOT (131-405 U/L), SGPT (51-Pragati Aditya Sathe* and Dipali Eknath Mahale Department of Pathology, Seth GS Medical College& KEM Hospital, Mumbai, India ABSTRACT Introduction:The spectrum of liver diseases in children differs from that in adults. Different diagnostic modalities which are available are not capable of diagnosing the liver disease individually unless they are supplemented by a liver biopsy. Hence liver biopsy is an invaluable tool in the diagnosis of liver diseases. Aims and Objectives:The aims of this study were to study the spectrum of liver diseases in pediatric age group at our institute, to correlate the liver biopsy findings with clinical findings and laboratory investigations and to review the literature on this topic. Results:We received a total of 130 liver biopsies during the five year study. Out of these, 65 biopsies were performed in children below one year of age while the remaining 65 liver biopsies belonged to children aged more than one year. The common indications for performing liver biopsies included prolonged jaundice, clay colored stools, abdominal distension, abdominal pain, suspected inborn error of metabolism and for a suspected hepatic tumor. Extrahepatic biliary atresia was the commonest pathology found in children below one year of age whereas metabolic disease was the most common pathology found in children above one year of age. Special stains helped in refining the diagnosis further. Laboratory investigations were correlated to offer a complete diagnosis. Conclusion:Paediatric liver biopsy is of value in diagnosis of disorders where clinical and laboratory parameters overlap. At the same time, clinical and laboratory correlation is a must due to overlapping histologic features of many liver diseases.

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“…Autoimmune hepatitis (AIH) is a chronic, immunemediated liver disease characterised by high serum transaminases, high immunoglobulin G (IgG) levels, detectable autoantibodies and histologically by the presence of interface hepatitis [84]. AIH is a rare condition both in adults and children, with a reported incidence of 0.4 and 3.0/100,000 children [85,86].…”

Section: Autoimmune Hepatitis and Autoimmune Sclerosing Cholangitismentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

Gupta

Samyn

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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Autoimmune hepatitis

Cited by 346 publications

References 220 publications

The Pathogenesis of Autoimmune Liver Disease

The Pathogenesis of Autoimmune Liver Disease

Pediatric Liver Biopsy: A clinicopathologic study

The management of childhood liver diseases in adulthood

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