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GOOD MORNING July 5th, 2013 Semantic Qualifiers Symptoms Acute /subacute Chronic Localized Diffuse Single Multiple Static Progressive Constant Intermittent Single Episode Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Recurrent Localized problem Systemic problem Abrupt Gradual Acquired Congenital Severe Mild New problem Recurrence of old problem Painful Nonpainful Bilious Nonbilious Sharp/Stabbing Dull/Vague Acute Colitis DDx** Infectious enterocolitis Pseudomembranous colitis (C. diff) Lymphocytic colitis Eosinophilic enterocolitis HSP HUS IBD Intestinal malignancies (Non-Hodgkin lymphoma) Colonoscopy Illness Script Predisposing Conditions Pathophysiological Insult Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) What is physically happening in the body, organisms involved, etc. Clinical Manifestations Signs and symptoms Labs and imaging IBD Epidemiology Mean age at diagnosis: 12.5 years Male: more likely pediatric Crohn’s disease Family history of IBD <20% diagnosed before 10y <5% diagnosed before 5 years Up to 25% of children who develop IBD w/ + family hx 1st degree relative with CD or UC = 10-13x higher risk! European or African descent Jewish ancestry Industrialized world Tobacco use: 2x increased risk Crohn Disease Epidemiology 3-5 per 100,000 30% of patients diagnosed before age 20 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 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 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 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 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 Clinical Manifestations** Oral exam for aphthous ulcers, as recurrent aphthousstomatitis also occurs in Crohn’s Disease.** Clinical Manifestations The following can also be seen on PE: Pallor Digital clubbing A benign abdomen Small for age 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 Serology IBD 7 tests for 7 markers of IBD Used to differentiate UC vs. CD ASCA and Anti-Omp C – specific for CD 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 Clinical Manifestations Label the picture as either Crohn Disease or Ulcerative Colitis Crohn Disease Ulcerative Colitis Ulcerative Colitis vs. Crohn** UC Crohn Rectal bleed Usual Sometimes Abdominal pain Common Common Malaise, fever, weight loss Common Common Perianal disease Rare Common Ileum involved None Common Strictures Rare Common Fistulas Rare Common Skip lesions - + Transmural - + Granulomas Rare Common Crypt Abscesses Usual Variable Risk of cancer ↑↑↑ ↑ Ulcerative Colitis vs. Crohn UC Crohn Cobblestoning - + Ulceration of IC valve - + Rectal sparing +/- + Crohn Disease can have eosinophila non-specific: h. pylori, EE, parasitic infections 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. 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 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 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 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 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 Treatment Proper nutrition Mediations guided by GI specialists Low residue diets or special formulas TPN if severe disease and malnourishment 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 Treatment Other medications Rifaximin - PO Antibiotic not absorbed Probiotics Check TMPT (thiopurine methyltransferase enzyme) Prior to starting 6-MP Alternative Therapy Helminth Marijuana Famous People with CD Noon conference: Thanks!!! Noon Conference!