So now let's go on to our next case.
A 32-year-old man presents to clinic to
follow up abnormal liver chemistry test.
His liver test we checked last month
to evaluate a 3-month history of fatigue.
He has a history of diarrhea for the past nine
months but has no known history of liver disease.
He has not had any abdominal
pain, fever or jaundice.
Vitals are normal.
His exam reveals hepatomegaly
but no splenomegaly.
There is no ascites,
jaundice or spider angiomata.
Labs are notable for an AST of 92 (U/L),
ALT 65 (U/L), alkaline phosphatase 475 (U/L)
and total bilirubin of 1.4 (mg/dL)
His abdominal ultrasound shows an enlarged
liver with thickened extrahepatic bile duct
but no intrahepatic
biliary dilatation or masses.
So what is the best next step in diagnosis?
Let's point out that he has a combination
of fatigue, with abnormal liver test
which should increase your suspicion
for some type of hepatobiliary disease.
He does also have a history of chronic
diarrhea which may be important a bit later.
In addition, on his physical exam and labs, he has
hepatomegaly and a cholestatic pattern of elevation.
The ultrasound shows abnormal extrahepatic bile ducts
but no other obvious abnormalities of the liver.
So that’s brings us to the diagnosis of
primary sclerosing cholangitis or PSC.
This is an inflammatory disorder
that affects the biliary system
which can be either
within or outside of the liver.
It tends to affect men more than women.
And will manifest with fatigue, a
generalized pruritus and jaundice.
Both of these are caused
by increased bilirubiin.
In addition, it can be
associated with complications.
So the disease may progress and
lead to cirrhosis, or scarring of the liver.
In addition, it is also associated
with a high risk of cholangiocarcinoma.
It is also associated with many autoimmune
diseases, in particular, ulcerative colitis.
And the diagnosis is made by looking for a
cholestatic pattern of injury on your liver test,
and performing an MRCP or
ERCP to confirm the diagnosis.
Unfortunately for this condition, there
is no really effective medical therapy.
What we can do instead is
perform endoscopic dilatation
of any strictures that are
formed in the biliary system.
And when the disease becomes very
severe, we can refer for liver transplantation.
So, we mentioned earlier that PSC has
certain risks of different types of cancers.
It increases your risk of cholangiocarcinoma
so patients who have this condition
have a 10-15% risk in their lifetime
of developing this type of cancer.
They are also at high risk for
hepatocellular cancer if their
disease progresses to cirrhosis
and other types of gallbladder cancer.
So, earlier we mentioned that you could
use an MRCP to make this diagnosis.
AN MRCP is magnetic resonance
Here on the left side you can see an
example of normal common bile duct anatomy.
So you can see a normal common bile
duct and normal hepatic ducts arising from it.
On the right side, this is an example of an MRCP
showing the typical "beaded ducts" appearance of PSC
so you see a very prominent and
irregular looking common bile duct
and irregular hepatic
ducts as is seen in PSC.
So now we return to our case.
A 32- year-old man coming in with
fatigue, known abnormal liver tests,
chronic diarrhea which potentially raises your suspicion
for inflammatory bowel disease such as ulcerative colitis
and a cholestatic pattern of elevation with
ultrasound showing abnormal extrahepatic bile ducts
but no other obvious abnormalities.
So at this point, you
should be suspecting PSC
and the best next step to confirm the
diagnosis is to do an MRCP or ERCP.
Thank you very much for your attention.