Celiac and Whipple Disease

1. Malabsorption
Syndromes
Shyala Chand
Group 3

2. Celiac Disease
• Chronic, auto immune inflammatory disorder
of the small bowel occurring in genetically
susceptible individuals.
• Caused by a reaction to Gliadin, a gluten
protein found in wheat, rye and barley
resulting in malabsorption.
• Female dominant, occurring at any age
(common in 1-5 years)

3. • Etiology:
 Environmental- Gliadin
 Immunologic- IgA antigliadin, antiendomysial, anti tissue
transglutaminase (tTG)
 Genetic- HLA-DQ2, HLA-DQ8
• Pathophysiology:
 Helper T cells mediate the inflammatory response.
 Absence of intestinal villi and lengthening of intestinal crypts
characterize the mucosal lesions in untreated celiac disease. More
lymphocytes infiltrate the epithelium (intraepithelial lymphocytes).
 Destruction of the absorptive surface of the intestine leads to a
maldigestive and malabsorption syndrome

4. Clincal Manifestation
• Gastrointestinal symptoms:
Diarrhoea- stools might be watery or
semiformed, light tan or gray, and oily or frothy
and have a characteristic foul odor.
Recurrent abdominal pain, distension, bloating
and cramping.
Malabsorption and anorexia.
Constipation
Lactose intolerance
Pancreatitis/ Hyposplenism

5. • Non GI symptoms
In females, may also cause miscarriages,
infertility and early menopause.

6. Diagnosis
• Lab Investigations:
Full blood count – anemia, features of hyposplenism
(target cells, spherocytes, Howell-Jolly bodies)
Biochemical tests- reduced calcuim, total protein,
albumin, Vit D, Vit B12 and Folic acid levels
Electrolytes
Prothrombin time - prolonged
Antibodies-

7. • Imaging:
 GOLD STANDARD- Endoscopic
small bowl biopsy.
 Findings:
 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

9. • Treatment:
 Correct existing deficiencies of iron, folate, calcuim and/or Vit D
 Lifelong Gluten-free diet
• Complications:
 Enteropathy associated T-cell lyphoma
 Small bowel carcinoma
 Squamous carcinoma of esophagus
 Ulcerative Jejunitis
 Osteomalacia, Osteoporosis
 Peripheral Neuropathy

10. 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

11. Whipple’s Disease
• Chronic, multisystemic disease caused by Gram +
bacillus Tropheryma whipplei.
• Commonly affects middle aged white males (>40 years)
• Pathophysiology:
Infilteration of bacteria within macrophages in bowel
mucosa.
 Villi are widened and flattened, containing densely
packed macrophages in the lamina propria, which
obstruct lymphatic drainage and cause fat
malabsorption.
 Patients with HIV infection do not acquire the disease.

12. Clinical Manifestations
• Opthalmoplegia

13. Diagnosis
• Lab investigations:
Full blood count- anemia due to
malabsorption
Stool Culture
Lumbar Puncture
Polymerase chain reaction- identification of
bacillus

14. • Imaging:
Small bowel biopsy- characteristic foamy
appearance of macrophages
MRI - assess brain involvment

15. • Treatment:
Fatal if untreated
Intravenous ceftriaxone (2mg daily for 2 weeks)
Oral co-trimoxazole for at least 1 year
• Complications:
Relapse (in 30% of patients with severe CNS
maifestations – treated with doxycycline and
hydroxychloroquine).
Severe deficiencies due to malabsorption.