2. DEFINITION
• An immune-mediated systemic disorder
• Elicited by gluten and related prolamines
• In genetically (mainly HLA) susceptible individuals
• Characterized by a combination of:
gluten dependent clinical manifestations,
anti-tissue transglutaminase (TG2) antibodies
and enteropathy
3. CLINICAL SPECTRUM
• Symptomatic CD
Frank clinical features(chronic diarrhea, failure to
thrive ,weight loss)
• Silent CD
No symptoms but +ve serology and HPE.
In most cases identified by serologic screening in
at-risk groups
4. • latent CD
- Normal HPE but before or after have
shown Gluten dependent enteropathy
Potential CD
Normal HPE but Positive serology .
It might or might not be symptomatic
5. Increased prevalence of CD in
children with
• Type 1 diabetes
• Autoimmune liver disease
• Autoimmune thyroid disease
• lgA deficiency
• Down's syndrome
• Turner syndrome
• Williams' syndrome
• First degree relatives with CD
2-12 %
12-13 %
up to 7 %
2-8%
5-12 %
2-5 %
up to 9 %
10-20 %
Garampazzi A, Rapa A, Mura S et al. Clinical pattern of celiac disease is still changing. J Pediatr
Gastroenterol Nutr. 2007;45:611-4.
6. Prevalent, But Under Diagnosed
- Those not diagnosed have a higher death rate
- Raise awareness
IMPROVE
SCREENING
7.
8.
9. Who Should Be Tested for CD?
Group 1: Group 2:
unexplained symptomsand signs
chronic or intermittent diarrhoea,
failure to thrive, weight loss,
stunted growth, delayed puberty,
amenorrhoea, iron-deficiency
anaemia, nausea or vomiting,
chronic abdominal pain, cramping
or distension, chronic
constipation, chronic fatigue,
recurrent aphthous stomatitis
(mouth ulcers), dermatitis
herpetiformis-like rash,
fracture With inadequate
traumas/osteopenia/osteoporosi
, and abnormal liver biochemistry.
Asymptomatic increased risk
forCD
Type 1 diabetes mellitus
(TlDM)
Autoimmune thyroid disease
Autoimmune liver disease
lgA deficiency
Down syndrome
Turner syndrome
Williams syndrome
First-degree relatives
10. DIAGNOSTIC TOOLS
• CD-specific Antibody Tests
• Histological Analysis of Duodenal Biopsies
• HLA Testing for HLA-DQ2 and HLA-DQ8
11. ANTIBODIES
• Anti-tTG /TG2 (tissue transglutaminase)
• Anti-EMA {Endomysial antibody}
• Anti- DGP ( deamidated Gliadin Peptides)
- lgA level should be done with EMA/tTG
• Anti- Gliadin/Anti- Reticulin
13. FALSE NEGATIVE SEROLOGY (tTG)
• Children younger than 2 yr
• Restricted gluten consumption
• Severe symptoms
• On immunosuppressive medications
• Technical problems
14. HISTOPATHOLOGY
• Crypt Hyperplasia
• Crypt to villous ratio is increased
• Intra-Epithelial Lymphocytosis
• Abnormal surface epithelium
• Subtotal to total villous atrophy
15. ENDOSCOPY & BIOPSY
Biopsies should be taken preferably
upper endoscopy
during
bulb {at least 1 biopsy)
second or third portion of duodenum {at least 4
biopsies)
20. Genetics
• Multiple genes involved
• The most consistent genetic component
depends on the presence of HLA-DQ
(DQ2 and/or DQ8) genes
• One or both of these genes are found in 95%
of celiac patients
• Having one or more of these genes doesn't
mean you will develop celiac, but if you have
the disease you likely have the gene.
-•
21. HLA TYPING
• HLA DQ2 and DQ8
- Strong negative predictive value
- Present in > 95 % patients of CD
- Present in > 30% normal population
- Weak positive predictive value
22. • Diagnosis without biopsy is still not recommended in
India & Developing countries
• Reliable HLA testing and EMA estimation is not widely
available
• Secondly tTG is being done by various labs with
different kits and their standardization is doubtful, so
>10 times criteria is also difficult to implement.
23.
24.
25. Symptoms
Present?
Yes
FOLLOW UP
CELIACDISEASE
Gluten Free Diet
Nutritional Education
Dietician
Periodic assessment af
Symptoms, Diet & Serology
Reinforce
adherence
Yes No
Evaluate for other causes
of symptoms
Consider Re- Biopsy
26. Management of Celiac Disease
Six key elements
• Consultation with a skilled dietitian
• Education about the disease
• Lifelong adherence to a gluten-free diet
• Identification and treatment of nutritional
deficiencies
• Access to an advocacy group
• Continuous long-term follow-up by a
multidisciplinary team
27. Treatment
Only treatment for celiac disease is a
gluten-free diet (GFD) < 20 ppm
- Strict, lifelong diet
- Avoid:
Wheat
Rye
Barley
30. GFD limitations
• Impairs quality of life
• Psychological impact
• Increase risk of obesity- overfeeding/low
fibre
• Altered eating pattern
• Chronic inadequacy of micronutrients
(low iron, Zn, Ca, Mg, vit D)
31. Treatment challenges
• Dietary compliance
• To get gluten free diet
• No reliable test- detect gluten in food
products
• No identification mark for GFD products
• GFD-more costly
• Lack of varieties
32. Barriers to compliance
Time pressure
Competing priorities - family, job, etc.
Feelings of deprivation
Social events
Assessing gluten content in foods - Label reading
33. Follow-up for dietary adherance
• Pretreatment values taken
• IgA levels fall earlier than IgG
• Either IgA TTG or AGA can be used
• Serological normalization precedes histological
recovery
• Measurement of TTG after 6 months of GFD-
decrease in antibody titer indicates recovery
• Persistent or recurrent symptoms with GFD - Rise in
antibody levels indicates dietary nonadherence
• Asymptomatic patient- measurement of TTG at
intervals of 1 year or longer may serve as a monitor of
adherence to the GFD
34. Follow-up
• Growth and maturation assessment
• Assessment for nutritional deficiency
• Counselling and psychological support
35. RESPONSE TO A GLUTEN-FREE DIET
90% IMPROVE (within 2 weeks)
10% FAIL TO IMPROVE
Dietary indiscretion
Lactose or fructose Intolerance
Microscopic colitis
Wrong Diagnosis
Pancreatic Insufficiency Bacterial overgrowth
Refractory sprue
36. CONCLUSION
• CD is common
• lgA tTG -good screening test for CD. ( exceptions
< 2 years)
• A gluten-free diet (GFD) should be introduced
only after the completion of the diagnostic
process, when a conclusive diagnosis has been
made{ expensive and lifelong diet).
