1. GOOD MORNING July 5 th, 2013

2. Semantic Qualifiers Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Localized problemSystemic problem AcquiredCongenital New problem Recurrence of old problem Symptoms Acute /subacuteChronic LocalizedDiffuse SingleMultiple StaticProgressive ConstantIntermittent Single EpisodeRecurrent AbruptGradual SevereMild PainfulNonpainful BiliousNonbilious Sharp/StabbingDull/Vague

3. Acute Colitis DDx**  Infectious enterocolitis  Pseudomembranous colitis (C. diff)  Lymphocytic colitis  Eosinophilic enterocolitis  HSP  HUS  IBD  Intestinal malignancies (Non-Hodgkin lymphoma)

4. Colonoscopy

5. Illness Script  Predisposing Conditions  Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc)  Pathophysiological Insult  What is physically happening in the body, organisms involved, etc.  Clinical Manifestations  Signs and symptoms  Labs and imaging

6. IBD Epidemiology  Mean age at diagnosis: 12.5 years  <20% diagnosed before 10y  <5% diagnosed before 5 years  Male: more likely pediatric Crohn’s disease  Family history of IBD  Up to 25% of children who develop IBD w/ + family hx  1 st degree relative with CD or UC = 10-13x higher risk!  European or African descent  Jewish ancestry  Industrialized world  Tobacco use: 2x increased risk

7. Crohn Disease Epidemiology  3-5 per 100,000  30% of patients diagnosed before age 20

8. Pathophysiology  Precise cause of IBD remains unknown  Genetic predisposition PLUS  Dysregulation between the immune system and the antigenic environment of the GI tract…which leads to GI inflammation and damage

9. Clinical Manifestations  What complaints would you expect a patient with UC to present with?**  Cardinal symptoms: diarrhea, rectal bleeding, and abdominal pain  Most present without systemic symptoms (fever, wt loss)  More severe presentation  Abdominal cramping associated with fecal urgency  Malaise  Low-grade/intermittent fevers  Anorexia with weight loss  Reflux or dyspepsia associated with upper GI inflammation

10. Clinical Manifestations  What complaints would you expect a patient with CD to present with?**  Classic presentation  Abdominal pain  Crampy, diffuse or RLQ  Diarrhea  Non-bloody, melanotic, or frank blood  Weight loss  Very important to plot height and weight in patients  Poor appetite, fevers, recurrent ulcers

11. Growth and IBD**  Growth failure may be the ONLY sign of IBD in 5% of patients. What are some causes of growth failure both before and after treatment is started?**  Occurs in 15-40% of children with IBD (CD > UC)  Reasons are multifactorial**  Food avoidance secondary to abdo pain/diarrhea  Increased cytokines  anorexia and growth hormone resistance  In Crohn Disease  Active inflammation of the small intestine  Decreases the intestinal surface absorption area  Causes protein-losing enteropathy + fat soluble vitamin deficiencies  Steroid treatment

12. Clinical Manifestations  Other than the abdomen, what important physical exam component MUST be assessed for disease?  Abdominal exam  Diffuse tenderness  Possibly RLQ tenderness or mass  Distension with more severe disease  Rectal exam…what might you see in a patient with CD versus UC?  CD: higher likelihood of fissures, skin tags, fistulas, and abscesses; can be an early indicator of disease**  UC: often normal

13. Clinical Manifestations**  Oral exam for aphthous ulcers, as recurrent aphthous- stomatitis also occurs in Crohn’s Disease.**

14. Clinical Manifestations  The following can also be seen on PE:  Pallor  Digital clubbing  A benign abdomen  Small for age

15. Work-Up**  What abnormal labs might you expect in a patient with IBD?  CMP:  albumin, possible  in transaminases,  Ca++  CBC: anemia of iron deficiency, B12/folate deficiency, or anemia of chronic disease  Elevated ESR and CRP  Fecal calprotectin and lactoferrin  Released by neutrophils that have migrated to the intestinal wall  Non-invasive markers of gut inflammation and can be elevated in other diagnoses  Abnormal IBD serologic panel

16. Serology  IBD 7  tests for 7 markers of IBD  Used to differentiate UC vs. CD  ASCA and Anti-Omp C – specific for CD

17. Work-Up**  An infectious cause should be eliminated before diagnosing IBD  Stool studies: Salmonella, Shigella, E. coli, Campylobacter, Yersinia, Giardia, Cryptosporidium  C. difficile toxin  PPD and Hepatitis test…should also be done before initiation of treatment with immunosuppressive Remicade  Upper GI, CT for complications, MRI  What is the “gold standard” for IBD diagnosis?  Endoscopy with biopsies

18. Clinical Manifestations  Label the picture as either Crohn Disease or Ulcerative Colitis  Crohn DiseaseUlcerative Colitis

19. Ulcerative Colitis vs. Crohn** UCCrohn Rectal bleed UsualSometimes Abdominal pain Common Malaise, fever, weight loss Common Perianal disease RareCommon Ileum involved NoneCommon Strictures RareCommon Fistulas RareCommon Skip lesions -+ Transmural -+ Granulomas RareCommon Crypt Abscesses UsualVariable Risk of cancer ↑↑↑↑

20. Ulcerative Colitis vs. Crohn  Crohn Disease can have eosinophila  non-specific: h. pylori, EE, parasitic infections UCCrohn Cobblestoning -+ Ulceration of IC valve -+ Rectal sparing +/-+

21. Extra-intestinal Findings  1/3 develop extra-intestinal manifestations, may occur before intestinal symptoms.  Your patient, who you suspect has IBD, also complains of stiffness and pain in his lower back. What do you suspect?  Ankylosing spondylitis  Is this more often associated with UC or CD? Ulcerative colitis  Which serum marker may be seen in this diagnosis? HLA-B27  Arthalgias and arthritis are common  Pauciarticular arthritis disease course correlates with intestinal disease activity.

22. Extra-intestinal Findings  Name 2 skin findings associated with IBD and tell which dx (CD or UC) it is more often associated with.  Erythema nodosum  More common in Crohn disease  Tender, warm, red nodules or plaques localized to the extensor surfaces  Pyoderma gangrenosum  More common in UC…up to 5% of pts  Associated w/ extensive colonic involvement  Lesions: discrete pustules with surrounding erythema  deep ulceration with well-defined border and deep color

23. Extra-intestinal Findings  Why would you want to consult ophthalmology upon diagnosis of IBD?  Risk of uveitis, episcleritis, corneal ulceration, and retinal vascular damage  Bone findings  Osteopenia  Osteoporosis  Decreased BMD seen in 25% of patients before steroids started  Aseptic necrosis

24. Extra-intestinal Findings  You are caring for a patient with known UC. His LFTs are elevated. He also complains of fatigue and anorexia. Mom feels like his eyes look yellow, and you notice him scratching throughout your exam. What is the most likely diagnosis?  Primary sclerosing cholangitis (PSC)  More common in UC patients  Increased GGT and Alkaline Phosphatase  Cholangiography and liver biopsy help confirm diagnosis  Increases risk of cancer

25. Nutritional Deficiencies  Crohn’s Disease  Anemia (folic acid and B12 deficiency)  Vitamin D deficiency  Hypocalcemia (related to low Vit, low albumin)  Zinc deficiency  Due to  Inadequate nutrition +/- poor absorption  Corticosteroid use

26. Admission  Severe Colitis  Fever  Hypoalbumnemia  Anemia  >5 bloody stools/day  Toxic megacolon  Occurs in up to 5% of adults with UC  Perforation may occur… very dangerous  Treatment upon admission  Bowel rest  TPN  IV steroids  Careful monitoring

27. Treatment  Proper nutrition  Low residue diets or special formulas  TPN if severe disease and malnourishment  Mediations guided by GI specialists  Corticosteroids (budesonide)  5-ASA (UC)  Immunomodulators (AZA, 6-MP, MTX)  biologic therapy, monoclonal Ab (Infliximab - Remicade)  Antibiotics (metronidazole, cipro for fistulas)  Surgery  For Crohn’s disease complications  For UC…total colectomy can be curative

28. Treatment  Other medications  Rifaximin - PO Antibiotic not absorbed  Probiotics  Check TMPT (thiopurine methyltransferase enzyme)  Prior to starting 6-MP  Alternative Therapy  Helminth  Marijuana

29. Famous People with CD Noon conference:

30. Thanks!!! Noon Conference!