ABSTRACT
Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating
non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in
the absence of a known etiology. Two types of childhood AIH are recognized according
to seropositivity: smooth muscle antibody (SMA) and/or antinuclear antibody (ANA),
which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1),
which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis
type 2 presents more acutely, at a younger age, and commonly with IgA deficiency;
however, duration of symptoms before diagnosis, clinical signs, family history of
autoimmunity, presence of associated autoimmune disorders, response to treatment,
and long-term prognosis are similar in the two groups. Immunosuppressive treatment
with steroids and azathioprine, which should be instituted promptly to avoid progression
to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to
nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate
mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1
patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis
with strong autoimmune features, including interface hepatitis and serological features
identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally.
The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing
cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive
treatment, whereas bile duct disease tends to progress.
KEYWORDS
Autoimmune hepatitis - sclerosing cholangitis - children
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Professor
Giorgina Mieli-VerganiM.D. Ph.D.
Paediatric Liver Centre, King's College Hospital, Denmark Hill
London SE5 9RS, United Kingdom
eMail: giorgina.vergani@kcl.ac.uk
