Coeliac Disease
Presented By:
Dr. Tanvi Patel
MD Repertory
Part I (Junior)
Introduction
It is an immunologiocally mediated
inflammatory disorder of the small
bowel occuring in genetically
susceptible individuals and resulting
from intolerance to wheat gluten and
similar proteins found in rye, barley and
oats.
It can result in malabsorption and
responds to a gluten-free diet.
The condition occurs worldwide, but is
more common in Northern Europe.
Coeliac disease is also known as
–
1. Coeliac sprue
2. Non-tropical sprue
3. Gluten-sensitive Enteropathy
Pathophysiology
Gluten peptides taken up by
epithelial cells.
Gluten peptides are deamidated (
converted to glutamic acid, which
leads to protein degradation) by
the enzyme tissue
transglutaminase in the
subepithelial layer.
Then they are able to fit the
antigen binding site on HLA-DQ 2
Recognition by CD4 + T cells
triggers a Th1 immune response
– generates pro-inflammatory
cytokines ( IL1, IFN-Y, TNF-a).
Lymphocytes infiltrate the lamina
propria, increase epithelial
lymphocytes, crypy hyperplasia
and villous atrophy.
Clinical Features
The classic clinical presentation
starts in infancy or early
childhood.
Chronic passage of 4-5 bulky,
paler, sticky, foul smelling stools.
The onset is generally preceded
by introduction of wheat or other
gluten containing cereals in the
diet for a couple of months or
more.
Growth Failure
Anaemia
Lack of playfulness
Multi-vitamin deficiecy
Diarrhea is due to – decrease
surface area for water and
electrolyte absorption, osmotic
effect of unabsorbed luminal
nutrients, and the stimulation of
intestinal fluid secretion by
inflammatory mediators and
unabsorbed fatty acids.
Other Extraintestinal
manifestations
Dermatitis Herpetiformis – crops
of intensely itch blisters over the
elbows, knees, back, buttocks.
Immunofluorescence shows
granular or linear IgA deposition
at the dermo-epidermal junction.
Treatment is mainly gluten-free
diet, but some require additional
treatment with Dapsone (100-150
mg daily).
Dermatitis Herpetiformis
Neurological disorders --
myopathy, epilepsy.
Psychaitric disorders --
depression, paranoia (mistrust).
Reproductive disorders --
infertility, spontaneous abortion.
Associated disease
HLA-linked autoimmune disorders
IDDM, Juvenile DM
Thyroid disease
Primary biliary cirrhosis
Sjogren’s syndrome – dry eyes
and dry mouth.
IgA deficiecy
Investigations
Duodenal or Jejunal Biopsy –
Endoscopic (Gold Diagnosis)
Villous atrophy
Crypt hyperplasia
Excess of interepithlial
lymphocytes
S. antigliadin (IgA), anti-
endomysial antibodies detected
by immunofluorescence.
A full blood count shows
microlytic or macrolytic anaemia
from iron or folate deficiency and
features of hyposplenism (target
cells, spherocytes, Howell-Jolly
bodies).
Biochemical tests may reveal
decrease concentration of
calcium, magnesium, total
Differential Diagnosis
Complications
Malignancy – lymphoma, CA of
GIT.
Endocrinal abnormalitis –
secondary hypopituitarism,
hypo-menorrhoea, amenorrhoea
or recurrent abortions in female
patients, hypogonadism,
oligospermia, abnormal sperm
motility.
Increase transaminases
Other Extraintestinal
Complications
Neuropathy
Myelopathy
Myopathy
Arthritis
Uveitisin eye
Splenic atrophy, etc.
Management
Gluten-free diet.
Exclusion of wheat, rye, barley
and oats.
Rice, maize and potatoes are
advised.
Dietary counselling.
Mineral and Vitamin
supplements.
Rarely, patient require
corticosteriods or
Prognosis
Ifpatient adhere strictly to a
gluten-free diet – Good prognosis.
Metabolic bone disease is
common in patient with long
standing, poorly controlled
coeliac disease.
Increase risk of malignancy.
References
1. Davidson’s Principles and
Practice of Medicine – 20th
edition.
2. API Textbook of Medicine – 6th
edition.
3. Cecil Textbook of Medicine
(Goldman Bennett).
Thank you !!
