Autoimmune liver disease

Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis

Autoimmune hepatitis (AIH) 386Autoimmune hepatitis/sclerosing cholangitis overlap syndrome (ASC) 388In paediatrics, two forms of autoimmune liver disease are recognized: • Autoimmune hepatitis (AIH)• AIH/sclerosing cholangitis overlap syndrome (autoimmune sclerosing cholangitis, ASC).• Progressive inflammatory liver disorder, preferentially affecting females, characterized serologically by high levels of transaminases and IgG and presence of autoantibodies, and histologically by interface hepatitis in the absence of a known aetiology....

2020 ◽  
Vol 07 (02) ◽  
pp. 11-14
Author(s):  
Sharon T Mathews ◽  

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.


Author(s):  
Satish Keshav ◽  
Alexandra Kent

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.


2017 ◽  
Vol 66 (1) ◽  
pp. S360
Author(s):  
M. Ferreira ◽  
C. Gonçalves ◽  
S. Nobre ◽  
S. Ferreira ◽  
I. Gonçalves

Hepatology ◽  
2017 ◽  
Vol 66 (5) ◽  
pp. 1570-1584 ◽  
Author(s):  
Rodrigo Liberal ◽  
Charlotte R. Grant ◽  
Muhammed Yuksel ◽  
Jonathon Graham ◽  
Alireza Kalbasi ◽  
...  

Digestion ◽  
2010 ◽  
Vol 82 (1) ◽  
pp. 24-26 ◽  
Author(s):  
Talha A. Malik ◽  
Alexandra M. Gutierrez ◽  
Brendan McGuire ◽  
Jessica G. Zarzour ◽  
Faisal Mukhtar ◽  
...  

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