Celiac disease Crohn s disease Ulcerative colitis Pseudomembranous colitis

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Aileen Booth
6 years ago
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1 2017 / nd semester/3rd practice Celiac disease Crohn s disease Ulcerative colitis Pseudomembranous colitis Semmelweis University 2nd Department of Pathology

CELIAC DISEASE = Gluten-sensitive enteropathy Autoimmune; frequent positivity of family history Higher frequency in the USA and Europe - 1% of people is affected Trigger: gluten NB!

2 CELIAC DISEASE = Gluten-sensitive enteropathy Autoimmune; frequent positivity of family history Higher frequency in the USA and Europe - 1% of people is affected Trigger: gluten NB! gluten does not present in nature it is produced by the person who mixes flour and water and makes the gluey ( glue ) complex (see.: bread moulding) Gluten = protein complex: glutenin & gliadin / secalin / hordein wheat (but ex. Scandinavia: 10%) Gliadin is hardly digestible/indigestible by digestive enzymes in most of the cases it leaves the body by the stool Getting into the lamina propria it is deamidated by TISSUE TRANSGLUTAMINASE enzyme: deamidated gliadin is an immunogen rye barley

PATHOGENESIS OF CELIAC DISEASE GLIADIN ZONULIN ttg TIGHT JUNCTION DISRUPTION LEAKY GUT DEAMIDATED GLIADIN ANTIGEN PREZENTING CELLS (APC) HLA-DQ2 / HLA-DQ8 CD4 + T-LYMPHOCYTES cytokines B-LYMPHOCYTES

3 PATHOGENESIS OF CELIAC DISEASE GLIADIN ZONULIN ttg TIGHT JUNCTION DISRUPTION LEAKY GUT DEAMIDATED GLIADIN ANTIGEN PREZENTING CELLS (APC) HLA-DQ2 / HLA-DQ8 CD4 + T-LYMPHOCYTES cytokines B-LYMPHOCYTES IgA > IgG CD8 + T-LYMPHOCYTES NKG2D-receptor direct tox. CITOTOXICITY ANTI-GLIADIN ANTI TISSUE TRANSGLUMATINASE (anti-ttg) ANTI - ENDOMYSIAL TG (EmA) ANTI - EPIDERMAL TG (anti-etg) N Engl J Med 2007; 357:

4 CELIAC DISEASE - HISTOLOGY INFLAMMATION CITOTOXICITY INTRAEPITHELIAL LYMPHOCYTOSIS VILLUS ATROPHY IEL REGENERATION CRYPT HYPERPLASIA

CELIAC DISEASE - MORPHOLOGY NORMAL CELIAC

5 CELIAC DISEASE - MORPHOLOGY NORMAL CELIAC (MARSHclassification) NORMAL CELIAC

6 CELIAC DISEASE - SYMPTOMS DIARRHEA, FATIGUE, WEIGHTLOSS DELAYED GROWTH ABDOMINAL PAIN METEORISM MOUTH, TONGUE SORES see SPRUE from the Dutch SPROUW (tongue sore) word HYPOSPLENIA Celiac disease MALABSORBTION Protein Fat steatorrhea Vitamin hypovitaminosis Iron, folic acid, B12 anemia D vit., Ca 2+ osteoporosis DERMATITIS HERPETIFORMIS in 15-25% of cases extremely itchy vesicles on the skin regions: elbow, knee, bottom, etc.

7 CELIAC DISEASE - SYMPTOMS DERMATITIS HERPETIFORMIS DUHRING in 15-25% of cases extremely itchy vesicles on the skin regions: elbow, knee, bottom etc. Journal of the American Academy of Dermatology, Volume 64, Issue 6, June 2011, Pages 1025 Wikimedia commons

8 CELIAC DISEASE - DIAGNOSIS SEROLOGY: ANTI TISSUE TRANSGLUTAMINASE (anti-ttg) ANTI - ENDOMYSIAL TG (EmA) ANTI - DEAMIDATED GLIADIN ANTI - GLIADIN IgA anti-bodies Celiac disease is often accompanied with IgA deficiency IgG has to be screened BIOPSY: GOLD STANDARD HAS TO BE DONE EVEN IF THE SEROLOGY IS POSITIVE AFTER GLUTEN CONSUPTION

9 CELIAC DISEASE IT CAN DEVELOP IN CHILDHOOD AND IN ADULTHOOD IT IS OFTEN ACCOMPANIED BY AUTOIMMUNE DISEASES TYPE I DIABETES MELLITUS AUTOIMMUNE THYROID DISEASES IgA DEFICIENCY LONG-TERM COMPLICATIONS RISK OF ADENOCARCINOMA IN THE SMALL INTESTINE RISK OF T-CELL MALT LYMPHOMA

10 CELIAC DISEASE - THERAPY GLUTEN FREE DIET Canadian Celiac Association, Gluten-Free Certification Program presentation, 2013

11 INFLAMMATORY BOWEL DISEASES (IBD) CROHN S DISEASE ULCERATIVE COLITIS COMMON FEATURES: GENETIC PREDISPOSITION IDIOPATHIC ETIOLOGY CHRONIC INFLAMMATION IN THE BOWELS ABNORMAL, DYSREGULATED IMMUNE RESPONSE SYSTEMIC DISEASES WITH BOWEL PREDISPOSITION EXTRAINTESTINAL MANIFESTATION UNDULATORY COURSE OF DISEASE

12 INFLAMMATORY BOWEL DISEASES (IBD) GENETIC FACTORS ANTIMICROBIAL RESPONSE NOD2 AUTOPHAGY ATG16L1 ER STRESS XBP1 CYTOKINES IL-23, IL-17 ENVIRONMENTAL FACTORS GUT FLORA DIET INFECTIONS NSAID SMOKING ANTIBIOTICS

Crohn s disease CROHN S DISEASE THE WHOLE GI TRACT CAN BE AFFECTED FROM THE ORAL CAVITIY TO THE ANUS IN 70% THE LARGE BOWEL IS AFFECTED SEGMENTAL PRESENCE OF THE DISEASE SKIP LESIONS TRANSMURAL

13 Crohn s disease CROHN S DISEASE THE WHOLE GI TRACT CAN BE AFFECTED FROM THE ORAL CAVITIY TO THE ANUS IN 70% THE LARGE BOWEL IS AFFECTED SEGMENTAL PRESENCE OF THE DISEASE SKIP LESIONS TRANSMURAL INFLAMMATION LINEAR ULCERS COBBLESTONE APPEARANCE DURING ENDOSCOPY FISSURES, FISTULES NON-CASEATING GRANULOMAS CRYPT ABCESSES segmental abscess SCARRING AND THICKENING OF THE BOWEL WALL STENOSIS OF THE LUMEN, STRICTURE CREEPING FAT Deep ulcer Fissure fistula perforation Stenosis of the lumen, fibrosis, transmural inflammation

15 CROHN S DISEASE EXTRAINTESTINAL MANIFESTATIONS

16 ULCERATIVE COLITIS Ulcerative colitis MAINLY PRESENTS IN THE COLON (+ so called. backwash ileitis can occur) CONTINOUS SPREADING FROM THE RECTUM TO PROXIMAL BOWEL PARTS THE INFLAMMATION IS RESTRICTED TO THE MUCOSA AND SUBMUCOSA BROAD-BASED ULCERS PSEUDOPOLYPS CRYPT ABSCESSES ATROPHY OF THE EPITHELIUM, METAPLASIA, DYSPLASIA pseudopolyp continuous TOXIC MEGACOLON (by reaching the plexus) DYSPLASIA CARCINOMA RISK!!! ulcer hemorrhage perforation Toxic megacolon

17 PSEUDOMEMBRANOUS COLITIS DUE TO DYSBACTERIOSIS AFTER ANTIBIOTIC TREATMENT SYMPTOMS: MUCOPURULENT, BLOODY, WATERY DIARRHEA; ABDOMINAL CRAMPING; FEVER MOST COMMON PATHOGEN: CLOSTRIDIUM DIFFICILE GRAM +, ANAEROBIC, TOXIN THE C. DIFFICILE COLITIS IS ONE OF THE MOST COMMON NOSOCOMIAL INFECTION 20% OF HOSPITALISATED PATIENTS HAVE C. DIFFICILE COLONISATION CONSEQUENCES: TOXIC MEGACOLON, PERFORATION TOXIN A: enterotoxin TOXIN B: cytotoxin

PSEUDOMEMBRANOUS COLITIS RISK FACTORS: ANTIBIOTIC USAGE HOSPITALISATION PPI, ANTIDEPRESSANT USAGE ELDERLY PATIENT WEAKENING OF IMMUNITY PREVIOUS DISEASES OF THE COLON: IBD, CRC ABDOMINAL

18 PSEUDOMEMBRANOUS COLITIS RISK FACTORS: ANTIBIOTIC USAGE HOSPITALISATION PPI, ANTIDEPRESSANT USAGE ELDERLY PATIENT WEAKENING OF IMMUNITY PREVIOUS DISEASES OF THE COLON: IBD, CRC ABDOMINAL SURGERY CHEMOTHERAPEUTIC TREATMENT HISTOLOGY: MUCOSAL ULCERS COVERED WITH FIBRINOUS EXUDATE, BETWEEN THEM INTACT PARTS CAN PRESENT SZÉP PÉLDA

19 PSEUDOMEMBRANOUS COLITIS

20 PSEUDOMEMBRANOUS COLITIS

Primary Care Update January 26 & 27, 2017 Celiac Disease: Concepts & Conundrums

Primary Care Update January 26 & 27, 2017 Celiac Disease: Concepts & Conundrums Alia Hasham, MD Assistant Professor Division of Gastroenterology, Hepatology & Nutrition What is the Preferred Initial Test