Juvenile autoimmune hepatitis: A comprehensive review

Sokollik, Christiane; McLin, Valerie A.; Vergani, Diego; Beretta-Piccoli, Benedetta Terziroli; Mieli-Vergani, Giorgina (2018). Juvenile autoimmune hepatitis: A comprehensive review. Journal of autoimmunity, 95, pp. 69-76. Elsevier 10.1016/j.jaut.2018.10.007

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Autoimmune hepatitis (AIH) is a rare, chronic disease that affects both adults and children, including infants.
The disease is probably triggered by environmental factors in genetically predisposed individuals. The clinical
presentation ranges from asymptomatic patients or patients with non-specific symptoms, such as fatigue, to
fulminant liver failure, many children presenting with symptoms indistinguishable from those of acute hepatitis.
Raised transaminase and immunoglobulin G (IgG) levels, in association with circulating autoantibodies, guide
towards the diagnosis. The histological hallmark is interface hepatitis, which however is non-specific and may be
absent. There are no bile duct changes on cholangiography. Presence of anti-nuclear antibody (ANA) and/or
anti-smooth muscle antibody (SMA) is characteristic for type 1 AIH, whereas presence of anti-liver kidney microsomal
type 1 (LKM1) antibody and/or anti-liver cytosol type 1 (LC1) antibody defines type 2 AIH. The latter
accounts for about one third of the juvenile AIH cases, presents more acutely than type 1 AIH and is very rare in
adults. Immunosuppressive therapy, based on steroids and azathioprine, is required, and in the vast majority of
patients leads to clinical and biochemical remission, defined as absence of symptoms, normal transaminase and
IgG levels, and negative or low-titer autoantibodies. In patients intolerant or non-responder to standard therapy,
a number of second line drugs have been employed with variable results. For the rare cases who progress to endstage
liver disease, liver transplantation is life-saving, but recurrence of the disease is possible. A better understanding
of the underlying pathogenic mechanisms will help to develop new, more effective and less toxic
therapies, and to tailor treatment regimens to the individual patient.

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