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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Clin+Transl+Hepatol
2015 ; 3
(1
): 42-52
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Update on Autoimmune Hepatitis
#MMPMID26357634
Liberal R
; Vergani D
; Mieli-Vergani G
J Clin Transl Hepatol
2015[Mar]; 3
(1
): 42-52
PMID26357634
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Autoimmune hepatitis (AIH), a liver disorder affecting both children and adults,
is characterized by inflammatory liver histology, elevated transaminase levels,
circulating nonorganspecific autoantibodies, and increased levels of
immunoglobulin G, in the absence of a known etiology. Two types of AIH are
recognized according to seropositivity: smooth muscle antibody and/or antinuclear
antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or
liver cytosol type 1 define AIH type 2. AIH type 1 affects both adults and
children, while AIH type 2 is mainly a paediatric disease, though it does
occasionally affects young adults. AIH should be considered during the diagnostic
workup of any patient with increased liver enzyme levels. AIH is exquisitely
responsive to immunosuppressive treatment with prednisolone with or without
azathioprine, with symptom free long-term survival for the majority of patients.
For those who do not respond to standard treatment, or who are
difficult-to-treat, mycophenolate mofetil and, in the absence of a response,
calcineurin inhibitors should be tried in addition to steroids. The pathogenesis
of AIH is not fully understood, although there is mounting evidence that genetic
susceptibility, molecular mimicry and impaired immunoregulatory networks
contribute to the initiation and perpetuation of the autoimmune attack. Liver
damage is thought to be mediated primarily by CD4 T-cells, although recent
studies support the involvement of diverse populations, including Th17 cells. A
deeper understanding of the pathogenesis of AIH is likely to contribute to the
development of novel treatments, such as the adoptive transfer of autologous
expanded antigenspecific regulatory T-cells, which ultimately aim at restoring
tolerance to liver-derived antigens.