BOF: 23
A 35-year-old female presented with a history of lethargy
and jaundice. She also complained of joint pains and mild pain in the right
upper quadrant.
She did not drink and did not take any medications
including over the counter preparations or herbal remedies.
On examination the patient was not obese, she had mild
jaundice and the liver was enlarged 3 fingerbreadths below the costal margin and
was tender.
Investigations revealed normal haematology raised ALT, ALP
and bilirubin.
Gammaglobulin levels were raised and she was positive for
ANA and SMA antibodies.
She was negative for hepatitis B and C viruses and
screening for haemochromatosis, Wilson’s disease and alpha antitrypsin
deficiency was negative.
Ultrasound scanning did not reveal a focal lesion.
Liver biopsy was performed and this showed a
lymphoplasmacellular infiltrate in the portal tracts with necrosis affecting the
limiting plate.
In this patient:
a)
Selective elevation of Ig G antibody is typical
b)
Selective elevation of Ig A antibody is typical
c)
Selective elevation of Ig M antibody is typical
d)
Positive anti SMA is diagnostic of this condition
e)
Positive anti LKM antibody is diagnostic of this condition
Answer:
a)
The clinical features point to
the patient having chronic hepatitis (lethargy, jaundice, pain in the right
hypochondrium hepatomegaly)
Alcohol and drugs are not the
cause as there is no relevant history.
Other common causes have been
excluded and what remain are elevated gammaglobulins and positive ANA and SMA
antibodies. This points to autoimmune hepatitis (AIH), which is confirmed by
liver biopsy.
The diagnostic criteria for AIH
are the following:
Major Criteria
Hypergammaglobulinaemia
(increased IgG)
Autoantibodies (ANA, SMA, SLA/LP,
LKM)
Absence of viral hepatitis
Portal hepatitis (with
lymphoplasmacellular infiltrate) on histology
Minor Criteria
Personal or family history of
other autoimmune disease
Spontaneously fluctuating course
Arthralgia
Presence of HLA-DR3 or DR 4
In AIH typically there are high
levels of IgG.
IgA suggests a toxic cause such
as ethanol
IgM is a feature of primary
biliary cirrhosis
ANA, SMA and LKM may be found in
other liver disease
SLA/LP antibodies are highly
specific for AIH and thus are diagnostic if detected (present in about 20%)
ANA antinuclear antibody
SMA smooth muscle antibody
LKM liver-kidney microsomal
antibody
SLA/LP soluble liver antigen
antibody
Last Updated: 13/04/06
