Today we'll discuss even further
disorders of the biliary system.
And we'll focus on cholestatic liver diseases.
So let's begin with a case.
A 65-year-old woman presents to clinic
with 4 months of fatigue, pruritus and dry eyes.
She does not have fever, chills, jaundice,
rashes, night sweats or weight loss.
She has a history of hyperlipidemia
for which she takes rosuvastatin.
She has no other medical problems.
Vitals are normal, physical
exam is unremarkable.
No rashes are noted.
Her labs are notable for an ALT of 75 (U/L),
AST 45 (U/L), alkaline phosphatase 420 (U/L)
and total bilirubin 1.5 (mg/dL)
In addition, her antimitochondrial
antibody titer is 1:640
A right upper quadrant ultrasound is normal.
What is the most likely diagnosis?
So let's point out here that she has
had 4 months of kind of vague symptoms
with generalized pruritis without a rash, which
would prompt you to think of a systemic disorder.
She also has a cholestatic pattern
elevation of her hepatic panel.
So, a primarily elevation in her alkaline
phosphatase and the total bilirubin
out of proportion to the ALT and AST.
In addition, she has this elevated
AMA antibody which we will discuss.
So, now let's discuss a condition
called primary biliary cholangitis.
This is an immune-mediated disease
where the small interlobular bile ducts
get destroyed by the autoimmune process.
It mostly affects middle-aged women
and can cause hypercholesterolemia
so patients will come in presenting
with fatigue, generalized pruritus
so a feeling of being itchy everywhere,
jaundice and dry eyes.
On physical exam, you may see evidence
of hyperpigmentation or darkening of the skin,
they may have xanthelasmas or xanthomas which are
findings of high cholesterol which we'll discuss later
and hepatomegaly or an enlarged liver.
The diagnosis is made by finding this cholestatic
pattern of elevation in the liver enzymes.
So again, that means a predominant elevation
in the alkaline phosphatase, GGT and bilirubin.
You may also confirm the diagnosis
with a positive antimitochondrial antibody.
So, we mentioned this physical exam
findings of xanthelasma and xanthoma.
A xanthelasma is simply a fatty cholesterol
deposit on the eyelid as shown here.
A xanthoma is a very similar finding except that it
is a deposit on the skin and usually over the joints.
So here you can see multiple
xanthomas overlying the joints of the hands.
So treatments of this condition involves
giving a medication called Ursodiol
to slow progression of the disease.
You will also treat them for their symptoms,
so antihistamines to treat their pruritus,
and artificial tears for their dry eyes.
And when the disease continue to progress, you
may refer for liver transplantation evaluation.
So now let's go back to our case.
Our 65-year-old woman who's coming in
with vague symptoms of pruritus, no rashes.
She has a cholestatic pattern
of elevation on her hepatic panel
and she has an elevated AMA antibody which should
prompt you to think of two particular diagnoses:
autoimmune hepatitis or
primary biliary cholangitis.
In her case, she does not have a
hepatocellular pattern of injury on her liver test
so the most likely diagnosis here, is PBC.
In addition, her positive anti-mitochondrial
antibody will help you confirm this diagnosis.