CELIAC DISEASE

objectives:

1- definition + risk factors

2- epidemiology

3- pathophysiology

4- clinical features

5- investigations

6- treatment

7- prognosis

definition : (gluten sensitive enteropathy)

is an inflammatory disorder of the small bowel occurring in genetically susceptible individuals, which results from intolerance to wheat gluten and similar proteins found in rye, barley and, to a lesser extent, oats.

risk factors:

Multifactorial

(genetic predisposition )

immunological response clinical

(environmental factors .. Trigger)

(intestinal mucosa)

Epidemiology:

*the incidence of celiac disease is estimated at 1%.

*but only small proportion has been diagnosed.

*most common in northern europe , no cases in china.

* Female : male (2:1).

pathophysiology

1-the patient eat something contain gluten.

2-in stomach the gluten break into ...

a-glutenin (easily digest)

b-gliadin (resist to digest in stomach)so go tn proximal part of duodenum

3-gliadin enter to lamina purpra by one of these three ways

paracellular (btween cells)

transcellular(through cells)

epithelial damage

4- gliadin deamidation by tissue transglutamimase to more negatively charge.

5-dendritic cells will recognize gluten as a foreign body and presents it is antigen through Major Histocompatibility Complex MHC II ,( mostly HLA-DQ2 and HLA-DQ8), this process will present gluten antigen to helper T-cells

6- T-helper cells have 3 fanfictions:

a- release cytokines that cause stress and damage epithelial cells

b-activation and prolifration of intaepithelial lymphocytes tjat kill epithelial cells

c-activation of B-cells that produce antibodes aginst gluten and tissue transglutaminase enzymes

7- make damage in mucosa (later on ).

why some persons have this disease and other not?

because there is variation in MHC II

clinical features

*The most common period of presentation is between 6 mo and 2 yr of age, but it can occur at any stage in life.

*clinically classified to 4 categories:

Typical: This presentation is primarily characterized by GI signs and symptoms.

Atypical: GI signs and symptoms are minimal or absent, and various extraintestinal manifestations are present.

Silent: The small intestinal mucosa is damaged, and celiac disease autoimmunity can be detected with serology; however, no symptoms are present.

Latent: Individuals with normal mucosal morphology but celiac disease autoimmunity can be detected with serology.

Typical and atypical :

typical presentation

1-chronic diarrhea(steatorrhea)... malabsorption of water

2-poor weight gain .... malabsorption of carbohydrate

3-abdominal pain,vomiting,constipation,anorexia,dehydration

4-tiredness .... malabsorbtion of electrolytes

5-FTT,delyed growth

6-deficiency in...

*iron and folic acid .. bimorphic anemia microcytic and macrocytic anima

*ca and vitamin D ... osteopenia, osteoporosis,rickets

*vitamin K .... bleeding

*vitamin B12...

*vitamin A.... night blidness

*protein ...edema and ascites

7- celaic crisis ...explosive watery diarrhea, marked abdominal

distension, dehydration, hypotension, and lethargy,

often with profound electrolyte abnormalities,

including severe hypokalemia

atypical presentation

Investigations: 1- serological markers:

Total IgA ….. begin investigate with it because it it low it affect other result (than patient have IgA deficiency)

Anti-Tissue Transglutaminase antibodies Anti-endomysial antibodies The antigliadin antibodies

2- The gold standard for diagnosing celiac disease is biopsy

Marsh classification …. multiple biopsies are taken during esophagogastroduodenoscopy (EGD) , Obtaining at least 4 biopsy samples from the distal duodenum and 1 or 2 from the bulb ishighly recommended because mucosal changes in celiac disease may be patchy.

** Important causes of subtotal villous atrophy (flat mucosa).

• Coeliac disease

• Tropical sprue

• Dermatitis herpetiformis

• Lymphoma

• HIV-related enteropathy

• Giardiasis

• Hypogammaglobulinaemia

• Radiation

• Whipple’s disease

• Zollinger–Ellison syndrome

•IBD

3-genetically screening

Genetic screening tests is not currently recommended , because the most common alleles found in celiac disease (HLA-DQ2 and HLADQ8) are common in general population , so their presence do not mean that they have the disease. but Negative findings for HLA- DQ2 and HLA-DQ8 can essentially rule out current or future celiac disease in patients for whom other tests, including biopsy, do not provide a clear diagnostic result

4- therapeutic trial (gluten free diet for 1 week)

5- Other laboratory studies should be performed to rule out complications , including : (CBC,Ca,PEI,LFT,total protein,vitamins,electrolytes)

TREATMENT •1- A strict, lifelong gluten-free diet is the only way to manage celiac disease. Besides wheat.

Barley

Bulgur

Durum

Farina

Graham flour

Malt

Rye

Semolina

Spelt (a form of wheat)

Triticale

Many basic foods are allowed in a gluten-free diet, including:

• Eggs

• Fresh meats, fish and poultry that aren’t breaded, batter-coated or marinated

• Fruits

• Lentils

• Nuts

• Potatoes

• Vegetables

2- take supplements ..(according to deficiency)

3- follow up…(clinically and serology )

4- If your small intestine is severely damaged or you have refractory celiac disease, your doctor might recommend steroids to control inflammation.

Steroids can ease severe signs and symptoms of celiac disease while the intestine heals.

Other drugs, such as azathioprine or budesonide.

PROGNOSIS

The prognosis is excellent; the disorder is fully reversible if trigger foods are avoided.

thank you