2. Definition:
• A chronic immunological mediated
inflammatory disorder that results in an
inability to tolerate GLIADIN ( The alcohol
soluble part of Gluten) leading to
malabsorption of nutrients.
• Gluten is found in wheat, Ryle and Barley
3. • Occurs in patients with HLA DQ2 and DQ8
• Affects 10 % of first degree relatives.
• The HLA molecules present Gliadin to T-cells
• The T Helper cells mediate the inflammatory
response.
• The endogenous Tissue Transglutamine
deamidates Gluten to Gliadine .
4. • Breast feeding / artificial milk???
• Time of introduction of Gluten during or after
weaning causes the disease in children at
risk???
5. Frequency:
• Underdiagnosed
• 1: 3000 of the population in USA
• Highest prevalence in Ireland and Finland,
may reach 1: 100. Hence may affect 1% of the
population
• Estimated 3 million in USA and 3 million in
Europe
• On the rise in Africa, Asia and Middle East
6. • Slightly more in females
• Bimodal age distribution
First 8-12 months
Second 3rd – 4th decade
• Mean age 8.4 years
• 20% of patients are older than 60 years
• Increased incidence in patients with type I
Diabetes, Down and Turner’s syndrome
7. Presentation:
• Classical Form:
• Atypical Form: Subtle symptoms
• Asymptomatic Celiac disease: Diagnosed by
screening in high risk patients.
8. GIT symptoms
• Diarrhea : 45-85%, might lead to dehydration,
electrolyte imbalance and metabolic acidosis.
• Steatorrhea:
• Flatulence and borborygmus
• Weight loss : 45%. In children failure to thrive and
growth retardation
• Weakness and fatigue: 80%
• Hypokalemia
• Abdominal pain
• 20-50% fulfil Rome criteria for IBS
9. Extra- Intestinal symptoms
Anemia: 10-15%.
Bleeding diathesis
Osteopenia and osteoporosis
Neurological symptoms: 14%, mainly due to
hypocalcemia.
Skin Disorder: Dermatitis Herpitiformis
( Purpuric papulo-vesicular skin eruption on the
extensor surfaces in 20% of patients especially the
elbows.)
10. • Hormonal disorders: Amenorrhea, impotence
and infertility
• Orthostatic hypotension
• Peripheral oedema
• Ecchymosis
• Cheilosis and glossitis
• Chvostik and Trousseau’s sign
• Bone disease and fractures
11. Diagnosis:
• The American College of Gastroenterology 2013
Guidelines
“ Patients should be tested prior to being placed on
Gluten free diet”
“ Antibody testing especially IgA Anti- Tissue
Transglutamine Antibody ( IgA TTG) is the best first
test, although biopsies are needed for
confirmation”
IgA- TTG : 95% sens. and 95% spec. in untreated CD
12. • Endomysial IgA
• Reticulin IGA
• Anti- Gliadin: Non specific
Patients with CD have a high incidence of total
IgA deficiency ( 3-5%)
So total IgA should be determined beforehand
If IgA deficient, IgG Transaminase can be
measured
13. • Electrolytes
• Hemoglobin and CBC
• Stool Exam
• Genetic testing
• Bone densiometry
• Oral tolerance tests eg. Lactose
• Albumin
• Calcium
• Alkaline Phosphatase
14. Upper Endoscopy
6 duodenal biopsies . Considered the standard
criterion to establish the diagnosis.
Endoscopic findings:
• Mucosal fold scalloping
• Reduced mucosal folds
• Mosaic pattern
Crossby’s capsule: For jejenal biopsies
15. Imaging
• Dilatation of the small intestine
• Coarsening or obliteration of the mucosal
pattern
• Fragmentation or flocculation of the barium in
the gut lumen
16. Histology
• Mucosa only involved
• Villi are atrophic or absent with decreased
villous to crypt ratio.
• Crypts are hyperplastic
• Proliferation of the plasma cells and
lymphocytes
17. Staging
• Stage 0: Normal NCGS
• Stage 1: Increased intraepithelial lymphocytes
more than 30% ( Non specific)
• Stage 2: Increased presence of inflammatory
cells and crypt cell proliferation with preserved
villous architecture.
• Stage 3: Mild (A), Moderate(B), Subtotal or total
(C) villous atrophy.
• Stage 4: Total mucosal hypoplasia
18. Treatment:
• Avoid Gluten : Excellent prognosis
• Refractory cases ( 5%) : Corticosteroids and/or
Azathioprine can be used with a poor
prognosis
19. Complications
Increased risk of malignancy:
• Adenocarcinoma of the :
- Oropharynx and oesophagus
- Pancrease , small and large bowel
- Hepatobiliary tract
• T-cell lymphoma and Non Hodgkin lymphoma.
