00:01
We're going to a second type
of autoimmune disease
causing damage to perhaps
eventually the liver.
00:09
And this, then brings us to
primary biliary cirrhosis.
00:11
First,
dissect the name.
00:15
Primary,
means to say there was no
predisposed condition
Biliary. With the biliary tree, you know
that it begins or anatomically it originates
in the liver.
00:28
Everything congregates
down by the bile duct
in zone 1.
00:31
And the bile duct then
starts to come out of the liver and
then start moving towards your
gall bladder.
00:37
There is every possiblity
that primary biliary cirrhosis
that it could be an interhepatic.
00:41
or extra hepatic disease.
00:43
if it is interhepatic
with primary biliary cirrhosis
then you are looking for
elevated liver function tests.
00:49
Here, you would find AMA.
00:51
Anti-mitochondria antibody which
I will show you in a second.
00:53
And there is every possibility
that you will have
extra hepatic manifestation
but because you have
the biliary tree involved,
understand that you will have an increase
in,
ALP, alkaline phosphatase.
01:07
Along with your liver function tests.
01:10
Affects women more so because
it is autoimmune.
01:13
Symptoms include pruritus and fatigue.
01:17
Pruritus is itching.
01:19
and often times in primary biliary
cirrhosis,
you can expect to find jaundice.
01:24
So, often times there is
jaundice,
and there is pruritus could give
you a couple of differentials.
01:30
Primary biliary cirrhosis is one of them.
01:32
I'll give you another one.
01:33
that often times students get
confused with
and that is Primary Sclerosing
cholangitis.
01:38
They sound quite alike and
if you are tired and fatigued,
and you are taking an exam and
you are on the 6th hour,
please be careful.
01:45
Your mind is thinking one thing
but your hand
might choose something else.
01:48
Be alert at all times.
01:50
Make sure that you choose
the answer that your mind
is actually thinking.
01:57
PBC. Primary biliary cirrhosis.
01:59
95% of your patient will be
AMA positive.
02:03
Anti mitochondrial Antibody
positive.
02:06
You are not going
to find this at all.
02:08
So, I'll make your life a little
bit easier
and as a differential
primary sclerosing cholangitis
we'll make sure we come up
with a differential but here
AMA positive
In primary sclerosing cholangitis,
AMA negative.
02:22
Here also, you'll find
elevated Alkaline phospatase
because the biliary tree
is involved.
02:28
if your biliary tree is affected,
I cannot effectively deliver,
bile to the duodenum.
02:35
O-oh!
What kind of food particles
are affected.
02:39
You must have
bile so that you can
metabolize lipid.
02:45
Right? What's that process called?
Emulsification.
02:49
If you don't have emulsification,
what kind of vitamins
do you think you are missing?
Duh! Fat soluble vitamins.
02:54
ADEK, A-D-E-K.
02:57
Why is osteoporosis here?
Which one of the fat soluble
vitamins, if it's defficient may then
lead into osteoporosis?
That is vitamin D deficiency.
03:09
So fat soluble vitamins have
been lost.
03:12
Or not properly
absorbed.
03:14
Upon biopsy,
You find granulomatous
infiltration of bile duct
granulomatous
infiltration of bile duct
Memorize that.
03:23
Know it.
03:25
Because in a little bit, when I talk
about primary sclerosing cholangitis,
Upon histology of
the bile duct,
You don't find granuloma,
We call this, "onion-skinning"
Management,
Ursodeoxycholic acide
and worst case scenario,
if it is a little bit too late,
and it's cirrhosis,
the only option at this point
will be liver transplantation.