2. •Celiac disease is an autoimmune disorder of the
small intestine that occurs in genetically
predisposed people of all ages from middle
infancy.
•Celiac disease is caused by a reaction to
gliadin, a gluten protein found in wheat, rye and
barley
•This condition has several other names
including:
celiac sprue,
non-tropical sprue,
endemic sprue,
gluten-sensitive enteropathy
3.
4. • The prevalence of clinically diagnosed
disease is 0.05–0.27%
• Prevalance in childeren 0.33 and 1.06%
• Prevalance in adults 0.18–1.2%
5.
6. • Celiac disease appears to be polyfactorial,
both in that more than one genetic factor
can cause the disease and also more than
one factor is necessary for the disease to
manifest in a patient.
7. 1. Genetics
• The vast majority of celiac patients have one of
two types of HLA DQ
• Two of these variants—DQ2 and DQ8—are
associated with celiac disease
• The reason these genes produce an increase in risk
of celiac disease is that the receptors formed by
these genes bind to gliadin peptides more tightly
than other forms of the antigen-presenting
receptor.
8. 2. Prolamins
• The majority of the proteins in food responsible
for the immune reaction in celiac disease are the
prolamins. These are storage proteins rich in
proline and glutamine
• Prolamins disrupt tight junctions between
enterocytes which allow large amino acids to enter
circulation and stimulate immune response
9. 3. Tissue Transglutaminase
• Anti-transglutaminase antibodies to the
enzyme tissue transglutaminase (tTG) are
found in an overwhelming majority of
cases.
• Tissue transglutaminase modifies gluten
peptides into a form that may stimulate the
immune system more effectively.
10. 4. Villous atrophy and
malabsorption
• The inflammatory process, mediated by T cells,
leads to disruption of the structure and function of
the small bowel's mucosal lining, and causes
malabsorption as it impairs the body's ability to
absorb nutrients, minerals and fat-soluble
vitamins A, D, E and K from food.
• Lactose intolerance may be present due to
the decreased bowel surface
reduced production of lactase but typically resolves
once the condition is treated
11. 5. Risk modifiers
• Infection by Rota virus
• Human intestinal adeno virus
• That smoking is protective against adult onset
coeliac disease
• Timing of the exposure to gluten in childhood is
an important risk modifier
• Prolonging breastfeeding until the introduction of
gluten-containing grains into the diet is associated
with a 52% reduced risk of developing celiac
disease in infancy
12.
13. 1. GIT
• Diarrhoea which is pale, voluminous and
malodorous
• Abdominal pain and cramping, bloatedness
with abdominal distention
• lactose intolerance
• adenocarcinoma and lymphoma of small
bowel
• Ulcerative jejunitis and stricturing
14. 2. Malabsorption-related
•
•
•
•
•
•
Weight loss
Fatigue
Anemia
Abnormal coagulation due to deficiency of vitamin K,
Bacterial overgrowth
Calcium and vitamin D malabsorption (and
compensatory secondary hyperparathyroidism) may
cause osteopenia (decreased mineral content of the
bone) or osteoporosis (bone weakening and risk of
fractures)
15. 3. Miscellaneous
• IgA deficiency
• an increased risk of infections and autoimmune disease
• Dermatitis herpetiformis; this itchy cutaneous condition has
been linked to a transglutaminase enzyme in the skin, features
small bowel changes identical to those in celiac disease and
occurs more often (in 2%) in patients with celiac disease.
• Epilepsy, ataxia (coordination problems), myelopathy,
peripheral neuropathy, and schizophrenia
• Growth failure and/or pubertal delay
• Miscarriage and infertility.
• Hyposplenism (a small and under active spleen)
• Other auto-immune disorders
diabetes mellitus type 1
autoimmune thyroiditis
primary biliary cirrhosis
microscopic colitis
18. Serologic Test
• Tissue transglutaminase (TTG) antibodies
• Antibodies against endomysium
• Antibodies against reticulin (ARA) or gliadin
(AGA)
• Guidelines recommend that a total serum IgA
level is checked in parallel, as coeliac patients
with IgA deficiency may be unable to produce the
antibodies on which these tests depend
21. • Most patients with celiac disease have a small
bowel that appears normal on endoscopies;
however, five concurrent endoscopic findings
have been associated with a high specificity for
celiac disease:
scalloping of the small bowel folds
paucity in the folds
a mosaic pattern to the mucosa -cracked-mud appearance
prominence of the sub mucosal blood vessels
a nodular pattern to the mucosa
22.
23. • The classic pathology changes of celiac disease in
the small bowel are categorized by the "Marsh
classification"
Marsh stage 0: normal mucosa
Marsh stage 1: increased number of intra-epithelial
lymphocytes, usually exceeding 20 per 100 enterocytes
Marsh stage 2: proliferation of the crypts of Lieberkuhn
Marsh stage 3: partial or complete villous atrophy
Marsh stage 4: hypoplasia of the small bowel architecture
24.
25. By diet
• Presently, the only effective treatment is a lifelong GLUTEN FREE DIET
• Rice, soyabean, potato and corn flour are safe
• No medication exists that will prevent damage, or
prevent the body from attacking the gut when
gluten is present.
• Strict adherence to the diet allows the intestines to
heal, leading to resolution of all symptoms in most
cases and, depending on how soon the diet is
begun, can also eliminate the heightened risk of
osteoporosis and intestinal cancer
26. Refractory Disease
This may be because
• The disease has been present for so long that
the intestines are no longer able to heal on
diet alone
• The patient is not adhering to the diet
• Because the patient is consuming foods that
are inadvertently contaminated with gluten
• In this case steroids and immunosuppresents
should be considered
27. Experimental treatments
• Genetically engineered wheat species, or wheat
species that have been selectively bred to be
minimally immunogenic
• A combination of enzymes (prolyl endopeptidase
and a barley glutamine-specific cysteine
endopeptidase (EP-B2)) that degrade the putative
33-mer peptide in the duodenum. This
combination would enable celiac disease patients
to consume gluten-containing products
28.
29. •
•
•
•
Intestinal T-cell lymphoma
Carcinoma of small intestine
Ulcerative jejunitis
Complications of nutritional deficiency
Anemia
Osteoporosis
Osteomalacia
Peripheral neuropathy
30.
31. • Prognosis is excellent if properly diagnosed
and treated
• In some patients it becomes refractory to
gluten withdrawal and it carries a poor
prognosis
• These patients may have developed
ulcerative jejunitis or T-cell
lymphoma(10% of patients)
32.
33. •
Regarding celiac disease all are true
except:
a) All patients of celiac disease have dermatitis herpetiformis
b) all patients of dermatitis herpetiformis have evidence of
celiac disease on intestinal biopsy
c) Dermatitis herpetiformis does not respond to gluten free diet
d) Drug of choice for dermatitis herpetiformis is Dapsone
Answer : a & c
34. •
In addition to celiac disease villous atrophy can
be caused by:
a.
b.
c.
d.
e.
Giardiasis
Lymphoma
Whipple disease
Both a and c
All of the above
Answer : e
35. •
All are true about celiac disease except:
a. Celiac disease is most commonly confused with
IBS
b. 10% of adults with iron deficiency anemia have
un diagnosed celiac disease
c. It is one of the most frequently diagnosed disease
d. Celiac disease not responding to gluten free diet
has poor prognosis
Answer : c