Transcript Celiac Disease (gluten-sensitive enteropathy) Introduction • Celiac Disease is a chronic disease of the digestive tract. • It interferes with the digestion and absorption.

Celiac Disease
(gluten-sensitive enteropathy)
Introduction
• Celiac Disease is a chronic disease of the digestive
tract.
• It interferes with the digestion and absorption of
food nutrients.
• People with celiac sprue cannot tolerate gliadin, the
alcohol-soluble fraction of gluten
• Celiac disease is caused by an autoimmune reaction
to gluten.
• The root cause of celiac disease is unknown, but
inheritance is a risk factor.
• Celiac Sprue is a lifelong disease, and if untreated it is
associated with increased mortality .
What Is Gluten ?
• “Gluten” is a general term for a composite of
the storage proteins gliadin and glutenin.
• These proteins (conjoined with starch) comprise 80%
of the total protein in wheat/rye/barley seed .
• Corn (maize), sorghum, and rice are considered safe
for a patient to consume. They do contain types of
gluten that do not trigger the disease
Frequency
• Approximately 3 million people in Europe and
another 3 million people in the United States are
estimated to be affected by celiac sprue.
• The highest prevalence of celiac sprue is in Ireland
and Finland and in places to which Europeans
emigrated, notably North America and Australia.
• The incidence of celiac sprue is increasing among
certain populations in Africa, Asia (India), and the
Middle East.
• Celiac disease can be seen first time in any
part of life.
• Usually, this disorder can be discovered in
childhood .
Pathophysiology
• Celiac Disease has a strong hereditary component.
• The prevalence of the condition in first-degree
relatives is approximately 10%.
• A strong association exists between celiac sprue and
two human leukocyte antigen (HLA) haplotypes (DQ2
and DQ8).
• Damage to the intestinal mucosa is seen with the
presentation of gluten-derived peptide gliadin,
consisting of 33 amino acids.
• Helper T cells mediate the inflammatory response.
• People with celiac disease have abnormally high
levels of associated antibodies like: anti-gliadin, which
unite with antigens (toxic amino acid sequences) that
are found in wheat, rye, and barley.
• In people with celiac disease, the immune system
treats gluten as a foreign invader and produces
elevated levels of antibodies to get rid of it, causing
symptoms and associated discomfort.
• As a result, intestinal villi gets damaged (shortening
and villous flattening)
How does gluten cause negative effects?
• Certain Pro- and Gln-rich gliadin peptide fragments
survive the digestion process & make it to the gut
• These peptides are deamidated by tissue
transglutaminase (tTGase)
• APCs in HLA-DQ2 or –DQ8 positive individuals
express these deamidated peptide fragments on
class II MHC molecules
• The resulting CD4+ T-cell mediated immune response
can eventually result in the development of celiac
disease
• Therefore, the only currently recognized
treatment for celiac disease is complete
abstinence from food grains containing gluten
proteins .
Risk Factors
• Having a risk factor for Celiac Disease makes the
chances of getting a condition higher but does not
always lead to Celiac Disease
• Celiac Disease can be associated with disorders such
as thyroid disease, Anemia of unknown cause,
type I diabetes or other autoimmune disorders.
• Family history of Celiac or autoimmune disorders.
• Race: Celiac sprue is most prevalent in Western
Europe and the United States. The incidence is
increasing in Africa and Asia.
• Sex: Incidence of celiac sprue is slightly higher in
females than in males.
• Age: The age distribution of patients with celiac
disease is bimodal, the first at 8-12 months and the
second in the third to fourth decades. The mean age
at diagnosis is 8.4 years (range, 1-17 y).
• Italian race : celiac is common in Italy and
descendents.
• Large amounts of gluten at weaning are associated
with an increased risk for developing celiac disease.
• Finally, repeated rotavirus infections in infancy
appear to be associated with a higher risk of
developing celiac disease autoimmunity in
genetically predisposed individuals
Histology of Celiac Disease
• The histological lesion of gluten sensitivity
primarily affects the proximal small bowel.
• the damage caused by celiac disease can be
more extensive than once thought, and that it
likely affects the entire small bowel, rather
than just the lamina propriaand crypt regions.
• The lesion is described as subtotal villus
atrophy.
The difference between normal (left) and abnormal (right) Mucosa
We can see counts of lymphocytes and plasma cells in lamina of damaged Mucosa
Symptoms
• Celiac Disease has wide spread symptoms.
• The list of signs and symptoms of Celiac
Disease includes about 35 symptoms .
• Symptoms in children may differ from them in
adults.
• The most common symptoms in adults are :
1-abdomenal pain
2-Diarrhea
3-Pale stools
4-Weight loss
5-Dehydration
• The most common symptoms in children are :
1-Delayed growth
2-irritability
3-Anemia
4-Weight loss
5-Bloating abdomen
Celiac Disease in Children
• Older children with celiac disease who present
with GI manifestations may have onset of
symptoms at any age.
• The variability in the age of symptom onset
possibly depends on the amount of gluten in
the diet and other environmental factors
Signs
• Physical findings depend on extent of celiac disease.
• Celiac disease may occur in asymptomatic individuals
without any positive clinical findings
• Bloating of the abdomen is a relatively common
finding .
• Dry mucosal membranes with vomiting or diarrhea
indicate the degree of dehydration.
• Some extraintestinal manifestations of celiac
disease can be shown in some patints.
• The most common signs we can see are:
1-weight loss
2-fluid retention
3-anemia
4-osteoporosis
5-bruising easily
6-peripheral neuropathy
7-infertility
8-muscle weakness
Complications
Dermatitis herpetiformis:
• A blistering skin rash that involves the elbows,
knees, and buttocks are associated with dermal
granular IgA deposits .
• Dermatitis herpetiformis is a rare occurrence in
childhood and is described almost exclusively in
teenagers and adults.
Dental enamel hypoplasia:
• These enamel defects involve only the permanent
dentition and may be the only presenting
manifestation of celiac disease.
• Often, GI symptoms are minimal or absent.
Short stature and delayed puberty:
• 10% of children with idiopathic short stature may
have celiac disease that can be detected on serologic
testing.
• Adolescent girls with untreated celiac disease may
have delayed onset of menarche.
Arthritis and arthralgia:
• Arthritis can be a common extraintestinal
manifestation of adults with celiac disease, including
those on a gluten-free diet.
• 3% of children with juvenile chronic arthritis may
have celiac disease.
Psychiatric disorders:
• celiac disease can be associated with some
psychiatric disorders, such as depression and anxiety.
• These conditions can be severe and usually respond
to a gluten-free diet.
Diagnosis
 First, a thorough physical examination is
conducted, including a series of blood tests.
Second, a duodenal biopsy is performed with
multiple samples from multiple locations in
the small intestine.
 And third,the gluten-free diet is implemented
• Physical Examination:
1-pallor (due to anemia)
2-hypotension (low blood pressure)
3-edema (due to low levels of protein, [albumin] in
the blood)
4-dermatitis herpetiformis (skin lesions)
5-easy bruising (lack of vitamin K)
6-loss of various sensations in extremities including
vibration, position and light touch (vitamin
deficiency)
7-signs of severe vitamin/mineral deficiencies which
may include:
-diminished deep tendon reflexes
-muscle spasms
• Serologic Tests
– EMA (Immunoglobulin A anti-endomysium
antibodies)
– AGA (IgA anti-gliadin antibodies) Some people do
not produce IgA antibodies.
– DGP (Deamidated gliadin peptide antibody)
– tTGA (IgA anti-tissue transglutaminase)
Biopsy
• It is the key of diagnosis.
• a biopsy of the small intestine [jejunal] is
called for.
• the tissue samples are examined under a
microscope for signs of injury
The biopsy can be taken by inserting a tube through the mouth and throat
Normal
Celiac Disease
• Under the microscope, we see the atrophy of
Mucosa and Elevated numbers of T-cell
lymphocytes .
• The small bowel biopsy samples of persons
with dermatitis herpetiformis often show
similar damage.
Prognosis
• The prognosis for patients with correctly diagnosed
and treated celiac sprue is excellent.
• The prognosis for patients with celiac sprue who are
not responding to gluten withdrawal and
corticosteroid treatment is generally poor.
• Mortality is usually associated with water and
electrolyte depletion.
Treatment
• There are two main ways to treat and control
celiac disease:
1- Diet
2- Corticosteroids
 Diet:
• Complete elimination of gluten-containing grain
products, which include wheat, rye, and barley, is
essential to treatment.
• After an initial period of avoidance, oats might be
reintroduced into the diet of patients with celiac
disease.
• These patients should be monitored carefully for
recurrent symptoms.
Avoid the gluten
Gluten-free food
These days you can find meals made
especially for celiac disease patints,
which have no gluten content.
Corticosteroids:
• Corticosteroids have anti-inflammatory properties
and cause profound and varied metabolic effects.
• These agents modify the body's immune response to
diverse stimuli.
• Prednisone: Can be used in patients with
refractory celiac sprue
 Adult:
30-40 mg/d PO; taper off completely in 6-8 wk
 Pediatric:
1 mg/kg/d PO; not to exceed 30 mg/d; taper off completely
in 6-8 wk
The End
By: Usef Bada