00:01
The clinical presentation.
00:03
So, the GI symptoms,
we'll come back
to the other
manifestations in a moment,
but the GI symptoms are going
to be exactly what you expect.
00:11
You are not absorbing nutrients,
you're going to have diarrhea,
it's going to be
the most common.
00:15
You will have steatorrhea.
00:17
So basically you're
not absorbing fat,
so you'll have floating stool
that is incredibly foul smelling.
00:24
There will be bloating
and flatulence.
00:26
That's because all those
nutrients that you are not absorbing,
the bacteria are metabolizing
and fermenting to make gas.
00:33
They'll make hydrogen gas,
they'll make CO2,
And then with that bloating,
with that basically gas
expanding the bowel that will
be felt as abdominal cramping.
00:46
The extra intestinal
manifestations
include things associated
with malnutrition.
00:51
You'll be tired,
there will be weight loss,
there will be muscle wasting.
00:55
Little kids will have so
called failure to thrive,
they won't hit their milestones
in terms of weight and height.
01:02
Because we are not
probably absorbing iron
and folate and
B12 appropriately,
there will be various forms
of anemia that are seen.
01:11
We won't be able
to absorb vitamin K,
that's a fat soluble vitamin.
01:16
And as a result of that we
may have a bleeding diathesis
because we're not
making the appropriate
procoagulant
factors in the liver.
01:25
Atrophic glossitis and
oral mucosal lesions
are due to folate
and B12 deficiencies
and can be seen
in other settings.
01:34
But it may be something
that occurs with celiac disease.
01:37
Peripheral cutaneous dermatitis herpetiformis may also be seen.
01:42
Making the diagnosis.
01:43
So a lot of this is clinical.
01:46
Yeah, I eat this food and
I have a heavy pasta dish
and I wash it all
down with beer.
01:53
And I feel terrible
for the next week.
01:57
Okay, that's a presumptive kind
of story that you will often hear
but things that you can
look for that it's gonna be
a little bit more specific perhaps,
you'll see edema.
02:06
Well, that's because you
are not synthesizing
albumin appropriately
because you are malnourished.
02:12
There will be
increased fecal fat.
02:14
That's actually a kind
of a pain in the rear end.
02:17
To accumulate that stuff,
you actually have to have the patient
collected for certain
period of time.
02:22
Your patients won't like that.
02:24
So you may have a
presumed fecal fat.
02:27
But it's going to be really
hard in most cases to get
your patients to go along
with the actual process.
02:33
You may have prolonged
coagulation times.
02:35
You're not making the
normal procoagulation factors,
because you're not
absorbing vitamin K.
02:42
A microcytic anemia
associated with iron deficiency
and macrocytic anemia associated
with folate or B12 deficiency.
02:48
And then we can
look specifically
for those biomarkers
that I talked about.
02:51
So anti transglutaminase
antibody,
the IGA antibodies
have that specificity
is reasonably
sensitive and specific
for making the diagnosis
of celiac disease.
03:05
Again, not causal of the
primary GI manifestations,
but are good biomarker
and probably are involved
in the peripheral cutaneous
dermatitis herpetiformis.
03:20
And then to nail it,
this is where pathology comes in.
03:24
Hurray for pathology.
03:25
We'll do a biopsy.
03:26
We will send a bioptome
all the way down through
the stomach through the
duodenum into the small bowel
and we'll take nips of tissue.
03:35
This will involve obviously
an upper endoscopy.
03:37
So the endoscopic features,
the mucosa looks atrophic.
03:41
There may be deeper fissures.
03:43
There are a whole variety
of things that we can see
if you're a trained endoscopist.
03:48
The histology is what you
should be paying attention to.
03:51
We're going to
see loss of the villi.
03:53
Remember that very
first slide where you know
we have beautiful villi
sticking up in the air
and then right next to it,
not any villi.
04:00
Same thing is seen on biopsy.
04:03
And so that really helps
in making the diagnosis.
04:06
We may also see that the
bowel is trying to compensate,
and we may see very deep crypts.
04:13
We will also see increased
intra epithelial lymphocytes,
inflammatory cells
that have been
activated and
recruited to this location.
04:21
And so the lamina propria may be
expanded with lots
of little blue dots.
04:25
If there is a normal
biopsy that pretty much
excludes the diagnosis
of celiac disease,
and then we have to start
thinking about other causes.
04:35
So managing this.
04:37
Get rid of the gluten.
04:40
It's kind of a pain.
04:42
But increasingly,
especially in developed countries,
you can find a lot of gluten
free pastas and other dishes
so that you don't have to quit
eating things that you enjoy.
04:57
Approximate 70% of patients
probably even more than that
will have clinical improvement
within a couple of weeks
once they go on
a gluten-free diet.
05:05
However, unfortunately,
there are about 5% of patients
who may not respond within
that 2-week period or ever
who have already had
significant damage to their GI tract,
and may have to be
treated in other ways.
05:20
We'll get to that in a moment.
05:22
Remember, a negative biopsy
pretty much rules this out.
05:26
So we need to consider
alternative diagnoses
and other things that
can manifest in very
similar fashion or
irritable bowel syndrome,
bacterial overgrowth,
a primary pancreatic insufficiency,
or an entity called
microscopic colitis,
where we have
increased inflammation,
again,
probably autoimmune in nature,
but not caused by
glutens or gliadins.
05:49
In settings, where we think
that there is an ongoing
and severe
inflammatory component,
we may consider
immunosuppression either
with glucocorticoids
or other agents.
06:01
The prognosis for those who
respond overall is excellent.
06:05
You just need to stay off
glutens and find alternatives.
06:09
For those who don't,
we have to consider
that ongoing inflammation
and ongoing injury to the
epithelium with proliferation
in the setting of all the
reactive oxygen species
and other inflammatory mediators
increases the risk for lymphoma
and increases the
risk for GI cancer.
06:28
So we have to then constantly
monitor those patients
who don't respond.
06:33
There is a variant or a
variation on the theme overall
of celiac disease called
collagenous sprue,
where as a result of the
ongoing inflammation,
we get a intense collagen
deposition within the lamina propria.
06:50
And that really significantly
impedes normal absorption.
06:53
So the epithelium
may actually be intact,
but we can't really get those
nutrients from the epithelium
into the lymphatics and
into the small vessels
that live in the lamina propria.
07:04
And so the patients will still
have severe malabsorption.
07:07
And with really
significant length of injury,
we can develop scarring
that will cause strictures,
so you can have
bowel obstruction.
07:18
With that we've reached
the end of an interesting
and important entity
for you to recognize
and that you will certainly see in
many of your patients celiac disease.