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Review article: biliary and hepatic involvement in IgG4-related disease.
[cholangiocarcinoma]
IgG
4
-
related
disease
(
IgG
4
-
RD
)
is
a
multi-systemic
disorder
.
IgG
4
-
related
sclerosing
cholangitis
(
IgG
4
-
SC
)
is
the
biliary
manifestation
of
the
disease
,
often
in
association
with
autoimmune
pancreatitis
(
AIP
)
.
Hepatic
manifestations
of
IgG
4
-
RD
are
less
well
described
within
the
literature
.
To
examine
and
present
an
overview
of
IgG
4
-
RD
with
a
focus
on
the
biliary
and
hepatic
manifestations
.
An
electronic
search
using
Medline
was
performed
.
Search
items
included
'
IgG
4
multi-system
disease
,
IgG
4
associated
cholangitis
,
IgG
4
associated
liver
disease
and
autoimmune
pancreatitis
(
AIP
)
'
.
IgG
4
-
RD
is
characterised
by
an
IgG
4
-
positive
lymphoplasmacytic
tissue
infiltrate
,
storiform
fibrosis
and
an
obliterative
phlebitis
.
The
HISORt
criteria
may
be
used
to
establish
the
diagnosis
and
incorporate
a
multi-disciplinary
approach
involving
histology
,
radiology
,
serum
IgG
4
levels
and
response
to
steroid
therapy
.
IgG
4
-
SC
is
the
commonest
extrapancreatic
manifestation
of
type
-
1
AIP
,
while
the
hepatic
manifestations
remain
poorly
defined
.
Important
differential
diagnoses
include
primary
sclerosing
cholangitis
,
secondary
sclerosing
cholangitis
,
cholangiocarcinoma
and
pancreatic
carcinoma
.
Current
treatment
regimens
remain
ill
defined
although
steroid
therapy
is
used
first
line
unless
contraindicated
.
Patients
with
relapsing
disease
or
multifocal
disease
should
be
considered
for
azathioprine
.
Available
data
would
also
suggest
a
role
for
rituximab
.
I
gG
4
-
related
sclerosing
cholangitis
is
a
common
manifestation
of
IgG
4
-
related
disease
which
requires
a
multi-disciplinary
approach
to
establish
the
diagnosis
.
Differentiating
IgG
4
-
related
sclerosing
cholangitis
from
other
conditions
,
both
benign
and
malignant
,
is
challenging
,
but
vital
.
Steroids
remain
the
mainstay
of
treatment
.
Our
understanding
of
the
pathogenesis
of
the
hepatic
manifestations
of
IgG
4
-
related
disease
continues
to
evolve
.
Diseases
Validation
Diseases presenting
"common manifestation"
symptom
cholangiocarcinoma
familial mediterranean fever
pyomyositis
systemic capillary leak syndrome
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