Celiac Disease.pptx

celiac disease ppt

1. CELIAC DISEASE Dr. Jan Muhammad Afridi Professor Deptt of Child Health KMC/MTI KTH

2. General Learning Outcomes: Learn the pertinent aspects of the history and physical exam relative to Celiac disease. To learn the presenting signs and symptoms of Celiac disease. Learn the patho -physiology ,diagnosis, differential diagnosis and treatment of Celiac disease.

3. Specific Learning Objectives By the end of this one hr lecture The Medical student will be able to: To define Celiac disease Pathogenesis of Celiac disease Genetics of Celiac disease Clinical manifestation Celiac disease Laboratory and histo -pathological findings Differential diagnosis Treatment of Celiac disease

4. Celiac Disease Celiac disease is an autoimmune disorder of the small intestine that occurs in genetically predisposed people of all ages from middle infancy. Celiac disease Is caused by a reaction to gliadin, a gluten protein found in wheat, rye and barley. This condition has several other names including : celiac sprue, non-tropical sprue, endemic sprue, gluten-sensitive enteropathy

5. Epidemiology The Prevalence Of Clinically Diagnosed Disease Is 0.05–0.27 % Prevalence in children 0.33 and 1.06% Prevalence in adults 0.18–1.2%

6. Pathophysiology Celiac disease appears to be polyfactorial , both in that more than one genetic factor can cause the disease and also more than one factor is necessary for the disease to manifest in a patient.

7. 1. Genetics The vast majority of celiac patients have one of two types of HLA DQ Two of these variants—DQ2 and DQ8—are associated with celiac disease The reason these genes produce an increase in risk of celiac disease is that the receptors formed by these genes bind to gliadin peptides more tightly than other forms of the antigen-presenting receptor.

8. 2 . Tissue Transglutaminase Anti-transglutaminase antibodies to the enzyme tissue transglutaminase ( tTG ) are found in an overwhelming majority of cases. Tissue transglutaminase modifies gluten peptides into a form that may stimulate the immune system more effectively.

9. 3 . Villous Atrophy & Malabsorption The inflammatory process, mediated by T cells, leads to disruption of the structure and function of the small bowel's mucosal lining, and causes malabsorption as it impairs the body's ability to absorb nutrients, minerals and fat-soluble vitamins A, D, E and K from food. Lactose intolerance may be present due to The decreased bowel surface. Reduced production of lactase but typically resolves once the condition is treated.

10. 4 . Risk Modifiers Infection by Rota virus Human intestinal adeno virus Timing of the exposure to gluten in childhood is an important risk modifier Prolonging breastfeeding until the introduction of gluten-containing grains into the diet is associated with a 52% reduced risk of developing celiac disease in infancy

11. Signs & Symptoms GIT: Diarrhoea which is pale, voluminous and malodorous Abdominal pain and cramping, bloatedness with abdominal distention lactose intolerance adenocarcinoma and lymphoma of small bowel Ulcerative jejunitis and stricturing Celiac Crisis and the Role of Steroid

12. 2. Malabsorption -related Weight loss Fatigue Anemia Abnormal coagulation due to deficiency of vitamin K, Bacterial overgrowth Calcium and vitamin D malabsorption (and compensatory secondary hyperparathyroidism) may cause osteopenia (decreased mineral content of the bone) or osteoporosis (bone weakening and risk of fractures)

13. 3. Miscellaneous IgA deficiency an increased risk of infections and autoimmune disease Dermatitis herpetiformis ; this itchy cutaneous condition has been linked to a transglutaminase enzyme in the skin, features small bowel changes identical to those in celiac disease and occurs more often (in 2%) in patients with celiac disease. Epilepsy , ataxia (coordination problems), myelopathy, peripheral neuropathy, and schizophrenia Growth failure and/or pubertal delay Miscarriage and infertility. Hyposplenism (a small and under active spleen)

14. Conti… Other Auto-immune Disorders diabetes mellitus type 1 autoimmune thyroiditis primary biliary cirrhosis microscopic colitis

15. DIAGNOSIS

16. Routine Lab Test Full blood count Electrolytes Calcium Vitamin B12 and Folic acid levels Prothrombin time

17. Serologic Test Tissue transglutaminase (TTG) antibodies Antibodies against endomysium Antibodies against reticulin (ARA) or gliadin (AGA) Guidelines recommend that a total serum IgA level is checked in parallel, as coeliac patients with IgA deficiency may be unable to produce the antibodies on which these tests depend

18. Endoscopy Endoscopic still of duodenum of patient with celiac disease showing scalloping of folds.

19. Histopathology The classic pathology changes of celiac disease in the small bowel are categorized by the "Marsh classification" Marsh stage 0: normal mucosa Marsh stage 1: increased number of intra-epithelial lymphocytes , usually exceeding 30 per 100 enterocytes Marsh stage 2: proliferation of the crypts of Lieberkuhn Marsh stage 3: partial or complete villous atrophy Marsh stage 4: hypoplasia of the small bowel architecture

20. Differential Diagnosis Cow milk protein allergy Giardiasis Tropical sprue Other malabsorption syndromes

21. Treatment By diet: Presently, the only effective treatment is a lifelong GLUTEN FREE DIET Rice , soyabean , potato and corn flour are safe No medication exists that will prevent damage, or prevent the body from attacking the gut when gluten is present. Strict adherence to the diet allows the intestines to heal, leading to resolution of all symptoms in most cases and, depending on how soon the diet is begun, can also eliminate the heightened risk of osteoporosis and intestinal cancer

22. 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 immuno-suppresents should be considered . Also Steroid is to be given in celiac crisis

23. Complications Intestinal T-cell lymphoma Carcinoma of small intestine Ulcerative jejunitis Complications of nutritional deficiency Anemia Osteoporosis Osteomalacia Peripheral neuropathy

24. Prognosis Prognosis is Excellent if properly diagnosed and treated In some patients it becomes refractory to gluten withdrawal and it carries a poor prognosis These patients may have developed ulcerative jejunitis or T-cell lymphoma(10% of patients)

25. COUNSELING