Autoimmune hepatitis

2018 ◽  
Author(s):  
Satish Keshav ◽  
Alexandra Kent
Keyword(s):  
Liver Disease ◽  
Natural History ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Autoimmune Liver Disease ◽  
Diagnostic Approach ◽  
Primary Biliary Cholangitis ◽  
Disease Etiology

This chapter discusses autoimmune liver disease, including autoimmune hepatitis (AIH), primary biliary cholangitis, and primary sclerosing cholangitis. It explores definitions of the disease, etiology, typical symptoms, uncommon symptoms, demographics, natural history, complications, diagnostic approach and other diagnoses that should be considered, prognosis, and treatment.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

scholarly journals Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in utah children: Epidemiology and natural history

Hepatology ◽  
2013 ◽  
Vol 58 (4) ◽  
pp. 1392-1400 ◽  
Author(s):  
Mark Deneau ◽  
M. Kyle Jensen ◽  
John Holmen ◽  
Marc S. Williams ◽  
Linda S. Book ◽  
...  
Keyword(s):  
Natural History ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis

scholarly journals Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

2020 ◽  
Vol 25 (2) ◽  
pp. 107-117
Author(s):  
Dong-Won Ahn
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Acute Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases, in which liver transplantation is required ultimately in most patients. Imaging studies such as magnetic resonance cholangiopancreatography have important role in diagnosis in most cases of PSC. PSC is usually accompanied by inflammatory bowel disease and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in endstage biliary cirrhosis requiring liver transplantation. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC and risk stratification is important to ensure all patients receive a personalised approach to their care. Medical therapy using ursodeoxycholic acid (UDCA) or obeticholic acid (OCA) has an important role to reduce the progression to end-stage liver disease in PBC.

scholarly journals Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Overlap Syndrome Resistant to Azathioprine and Intolerant to Tacrolimus with Sepsis Mimicking Flare UP-A Clinical Dilemma

2020 ◽  
Vol 07 (02) ◽  
pp. 11-14
Author(s):  
Sharon T Mathews ◽  
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Kidney Injury ◽  
Scoring Systems ◽  
Overlap Syndrome ◽  
Rare Presentation ◽  
First Line Therapy ◽  
Progressive Liver Disease

Patients with autoimmune liver disease in a frequency of 7% -18% fit into the diagnostic criteria for more than one condition. Up to 12.5% of Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC) cohorts have a label of AIH/PSC overlap. There can be an unpredictable interval of many years between the diagnosis of the two conditions. There are scoring systems but no established criteria for the diagnosis of AIH/PSC overlap. Therefore, a diagnosis based on combination of biochemistry, autoantibody profile, cholangiogram, and liver histologyis made. Histopathology staging of AIH/PSC overlap influences therapeutic options and prognosis. There is beneficial role of immunosuppression, even though there is a higher relapse rate and evidence of progressive liver disease despite immunosuppression in some cases. Liver related outcomes in this overlap are better than PSC alone but are poorer than AIH. We herein report a rare presentation of an overlap syndrome with AIH and PSC in a patient in whom, treatment with first line therapy showed incomplete response and the salvage therapy was not tolerated, presenting with Acute Kidney Injury (AKI) and a clinical picture of sepsis resembling AIH flare.

Autoimmune Hepatitis/Overlap Syndromes

2018 ◽  
Author(s):  
Albert J Czaja
Keyword(s):  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Clinical Evidence ◽  
Calcineurin Inhibitors ◽  
Primary Biliary Cholangitis ◽  
Histological Findings ◽  
Overlap Syndromes ◽  
Inflammatory Bowel ◽  
Lymphoplasmacytic Infiltration

When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.  This review contains 6 figures, 7 tables and 50 references Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features

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