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Padhy
P.K.,
Barik,
B.K.,
Padhan
P.,
Pathak
R.C.,
Tripathy
S.,
Tripathy
S.
Abstract:
A
rare
case of
cirrhosis
of liver
following
autoimmune
hepatitis
is
reported
in a
young
female
patient.
The
pathophysiology,
clinical
presentation
and
management
of this
condition
are
reviewed.
Key
Words:
Autoimmune,
cirrhosis,
hepatitis
Introduction:
Autoimmune
hepatitis
(formerly
called
autoimmune
chronic
active
hepatitis)
is a
chronic
disorder
characterized
by
continuing
hepatocellular
necrosis
and
inflammation,
usually
with
fibrosis,
which
tends to
progress
to
cirrhosis
and
liver
failure.
The
prominence
of
extrahepatic
features
of this
disorder
supports
an
autoimmune
process
in its
pathogenesis.
Because
autoantibodies
and
other
typical
features
of
autoimmune
disorder
do not
occur in
all
cases, a
broader
more
appropriate
designation
of this
type of
chronic
hepatitis
is
idiopathic
or
cryptogenic.
Case
Report
A 33
year old
female
patient
came to
us with
history
of
amenorrhoea
for last
6
months,
gradual
distension
of
abdomen
for last
3
months,
one
episode
of
hematemesis
2 months
back and
scanty
urination
for 10
days.
One
month
prior to
admission,
the
patient
was
diagnosed
provisionally
as a
case of
cirrhosis
of liver
and
sclerotherapy
was done
for
bleeding
esophageal
varices.
Patient
was on
regular
treatment
with
propranolol
40mg
/day,
Pantoprazole,
Sucralfate
and
other
supportivce
drugs.
Patient
had no
further
hematemesis
after
sclerotherapy,
but
symptoms
of
progressive
liver
failure
persisted.
She is
not a
known
case of
DM, HTN,
SCD,
Pul, TB.
There is
no
history
of
intake
of
heparotoxic
drugs or
joint
pain. On
examination,
the
patient
was
edematous
with
mild
pallor,
mild
icterus,
with a
pulse
rate of
68/min,
regular,
BP
110/90
mm Hg,
CVS-NAD,
chest-bilateral
basal
fine
crepitions,
abdomen-moderate
ascites
with
engorged
abdominal
veins,
the 24
hr urine
output
was
100ml.
Investigation
Hb% -
8.4%,
TLC
7,900/mm3,
DC-N
80%, E
6%,
L-14%
RBS
104m%
Urine
Sugar
Nil,
Albumin
- +
Sr. urea
5-6%,
Sr.
creatinine
2mg%
Sr. Na+
- 100
mmol/lt,
Sr. K+
- 2.0
mmol/lt
Sr. RA
factor
Negative
HbsAg
Negative
LE cell
Negative
Anti
nuclear
antibody
positive
(Odd
ratro
1.64),
(+ve>1.0)
Anti ds
DNA
Borderline
positive
LFT:
S.
bilirubin
1` -
0.72mg%
S.
bilirubin
30` -
2.11mg%
SGPT
25IU/lt
SGOT
62 IU/lt
Sr. Alk.
Phosphate
135
IU/lt
Sr. GGT
12.2
IU/lt
Sr. Alb
1.7
gm/dl
Sr.
Protein
6.5gm/dl
A: G
ratio
0.35
USG of
abdomen
Ascites
with
splenomegaly,
dilated
portal
vein
Chest X
ray PA
view
NAD
Needle
biopsy
of liver
showed
portal
and
periportal
mononuclear
infiltrate
with
areas of
piecemeal
necrosis
and
fibrosis.
The
patient
was
diagnosed
as a
case of
autoimmune
cirrhosis
of
liver,
started
on oral
prendisolone
30mg/day
and
other
supportive
treatment.
The
patient
showed
marked
improvement
after
starting
oral
prednisolone.
The
condition
of the
patient
was
found to
be
stable
on
regular
follow
up since
last six
months.
Discussion:
Autoimmune
hepatitis
(AIH)
an
inflammatory
liver
disease
of
unknown
etiology
characterized
by
suppressor
T cell
defects
and the
production
of
autoantibodies
directed
against
hepatocyte
surface
antigens.
Two main
types
are
recognized
according
to the
presence
of
circulating
autoantibodies.
Type I
After
adults
or
childredn
antinuclear
antibodies
(ANA)
and / or
Antismooth
muscle
antibodies
(SMA)
Type II
Affect
children
Anti
liver/kidney
microsomal
type I
(LKM I)
antibodies.
Clinical
Features
Predominantly
affects
young
and
middle
aged
women,
25%
present
with
acute
hepatitis
and
features
of an
autommune
disease
e.g.
fever,
malaise,
utticarial
rash,
polyrthritis,
pleurisy
or
glumerulonephritis.
The
remainder
present
insidiously
or are
symptomatic
and
diagnosed
incidentally
with
signs of
chronic
liver
disease.
Amenorrhoea
is
common.
Associations:
·
Autommune
thyroiditis
·
Autoimmune
hemolytic
anemia
·
Penicious
anemia
·
Diabetes
mellitus
(Type I)
·
Ulcerative
colitis
·
Glomerulonephritis
·
HLA AI,
B8 and
DR3
haplotype
Tests
·
Abnormal
LFT (AST)
·
Hypergammaglobulinemia
·
+ve
for
autoantibodies
(ANA,SMA
orLKM)
·
Anemia
TIC &
TPC (Hypersplenism)
·
Liver
biopsy :
Mononuclear
infiltrate
of
portal
and
periportal
areas
with
piecemeal
necrosis,
fibrosis
and
other
features
suggestive
of
cirrhosis.
·
An ERCP
should
be
performed
to
exclude
primary
sclerosing
cholangitis
if
alkaline
phosphatase
is
disproportionately
raised.
Treatment
Immunosuppression
(prednisolone,
azathioprine
)
Liver
transplantation,
but
recurrence
may
occur
Prognosis
: There
is
paucity
of
literature
about
autoimmune
hepatitis
among
Indian
population.
The
disease
however
shows
variable
natural
history.
Mild
cases
are
marked
by
remissions
and
exacerbations,
response
to
immunosuppression
being
excellent.
Overall
mortality
without
treatment
is as
high as
40%.
Moderate
to
severe
cases
progress
to
cirrhosis
of
liver,
hepatic
failure
and
rarely
hepatocellular
carcinoma.
In
general,
immunosupression
remains
the
mainstay
of
treatment
and
treatment
often
slows
down the
disease
process.
Hence
early
diagnosis
and
institution
immunosuppression
is
rewarding.
Conclusion
The
diagnosis
of
autoimmune
hepatitis
requires
a high
degree
of
suspicion,
thus
many
cases
remain
undiagnosed.
All
cases of
hepatocellular
failure
where no
obvious
cause
like
viral
hepatitis,
alcoholism,
ingestion
of
hepatotoxic
drugs
etc. are
found
should
be
investigated
for
autoimmune
hepatitis.
References:
1.
Krawitt
E.L:
Autoimmune
hepatitis.
N. Engl.
J. Med.
334;897,1996.
2.
Czaja
A.J. et
al.
atommne
hepatitis;
Evolving
concepts
and
treatment
strategies.
Dig.
Dis.
Sci. 40;
435,1995.
3.
Czaja
A.J. et
al.
Associations
between
alleles
of the
major
histocompatibility
complex
and type
I
autoimmune
hepatitis,
Hepatology
25; 317,
1997.
Johnson
P.J. et
al.
Meeting
report;
International
autoimmune
hepatitis
group.
Hepatology
18:998,1993. |
