IBD Case of the Month: Oral Aphthous Ulcers in a Pediatric Patient

1. IBD Case of the Month:Oral Aphthous Ulcers in a Pediatric Patient Developed by the Crohn’s &colitis foundation Nurse &Advanced Practice Committee Author: Kristin Madden, MSN, APRN-PNP Children’s Hospital & Medical Center, Omaha, NE

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3. Objectives • Recognize extra-intestinal manifestations of Inflammatory Bowel Disease (IBD) • Identify association of oral aphthous ulcers to workup of IBD

4. Introduction/Background KO a 17 year old male presents to the pediatric GI clinic with: • Abdominal pain x 2 years – intermittent and able to be ignored • Nausea but no vomiting x 3 months • Decreased appetite with 10 lb weight loss in the last 2 months • Oral aphthous ulcers x 2 months • Fatigue

5. What additional information will be helpful? • Family History • Labs – what would you order and why? • Physical Exam • Previous Workup – none available

6. Review of Systems (ROS) General: fatigue, decreased appetite/early satiety, weight loss, NO fever Skin: negative ENT: mouth ulcers Respiratory: negative Cardiovascular: negative GU: negative GI: negative Musculoskeletal: negative Hematologic: negative Neurologic: negative Endocrine: negative Psychosocial: negative

7. Do you have red flags/cause for concern based on physical exam & review of systems? • No concern – follow up in GI PRN • Only minimal concern – no need to order further workup but return to clinic in 2-3 month to follow-up • Significant concern – requires further workup today • Major concern indicating need for admission

8. Do you have a Differential Diagnosis? • Peptic Ulcer Disease • Celiac Disease • GI Bleed • Infection • Post-viral Gastroparesis • Ulcerative Colitis • Crohn Disease • Lactose intolerance • Pancreatitis

9. What would be ordered for workup and what are results?(In this order) • Labs • Esophagogastroduodenoscopy and Colonoscopy with biopsies • MRE • Labs at time of endoscopy (when endoscopic appearance supports diagnosis of Crohn’s Disease)

10. What is your plan of care? • Oral Prednisone • 6MP • DEXA • Hepatitis B re-immunization • High calorie diet – avoid lactose

11. Summary Not all cases of IBD are reflective of bloody stools or diarrhea. It is important to recognize the extra-intestinal manifestations of IBD and if they resolve once treatment has been initiated. _______________________________________________________________ Most common extra-intestinal manifestation of IBD include:

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13. Oral Aphthous Ulcer back

14. Family History • Maternal Grandfather – Crohn Disease, colonic polyps • Maternal grandmother – hypothyroidism • Mother – Irritable bowel syndrome back

15. Growth Curve WAZ-score = -0.82 BMI Z-score = -1.74 back

16. What labs to order? • CBC – evaluate anemia • TIBC/Iron – evaluate iron deficiency • Ferritin – evaluate ferropenia, may be falsely elevated as it is an acute phase reactant with inflammation • CMP – evaluate liver enzymes, electrolytes and hypoalbuminemia • ESR/CRP – evaluate inflammation • Celiac Serology – rule out fatigue and weight loss as result of celiac disease • TSH/Free T4 – rule out fatigue and weight loss as a result of thyroid dysfunction • Amylase/Lipase – get baseline and rule out pancreatitis back

17. IBD and Lactose Lactose intolerance is a problem in the digestive tract caused by too little production of the enzyme lactase. The enzyme lactase is responsible for breaking down milk sugars and without it symptoms can range from mild to severe. Those symptoms may include: intestinal cramps, bloating, nausea, gas and diarrhea. With Crohn’s Disease patients may develop secondary lactose intolerance. This is thought to be due to the damage Crohn’s can cause to the intestinal lining. The intestines are the source of the production of lactase and when damaged may no longer be able to produce enough of the enzyme. back

18. Physical Exam General: alert, cooperative, no distress Head: normal Lungs: clear bilaterally Cardiovascular: regular rate, rhythm, no murmur Abdomen: soft, non-distended, no organomegaly, normal bowel sounds, no masses, epigastric tenderness Rectal: deferred with plan to scope Extremities: normal Neurologic: alert and developmentally appropriate Skin: no rash, no petechiae, no jaundice back

19. No Concern Incorrect – weight loss, fatigue and mouth ulcers indicate an underlying gastrointestinal disease may be present. Workup should be ordered today in clinic. back

20. Minimal Concern Incorrect – there is recognizable need for ordering labs to make sure there is no anemia or electrolyte abnormalities and evaluate for elevated inflammatory markers back

21. Significant Concern Correct – We need to move forward with urgent/not emergent workup to look for anemia, elevated inflammatory markers, hypoalbuminemia or electrolyte abnormalities. We need to prevent growth failure and/or stunted growth by not allowing a chronic inflammatory process to progress without appropriate workup or treatment since patient presented with a 10 lb weight loss back

22. Major Concern Incorrect – there are no emergent factors that we are aware of indicating need for admission to the hospital back

23. Results back

24. EGD/Colonoscopy Histology Endoscopic appearance Duodenum, biopsy:Acute duodenitis. Stomach, biopsy:Mild chronic inactive gastritis. Esophagus, distal, biopsy:Esophagitis with 0-8 eosinophils/hpf. Esophagus, proximal, biopsy: Portions of esophageal squamous mucosa with scattered lymphocytes and rare eosinophils. Terminal ileum, biopsy:Ulcerative ileitis. Ascending colon, biopsy: No histopathologic abnormality. Transverse colon, biopsy: No histopathologic abnormality. Descending colon, biopsy: No histopathologic abnormality. Rectosigmoid colon, biopsy:No histopathologic abnormality. back

25. Magnetic Resonance Imaging(MRE) MR enterography is a special type of magnetic resonance imaging (MRI) performed with a contrast material to produce detailed images of the small intestine. Provides intraluminal and extraluminal imaging for best decision making. This will evaluate extent of small intestinal involvement and identify any strictures. KO’s MRE – 1. Continuous distal ileal bowel wall thickening and hyperemia, compatible with a nonspecific infectious or inflammatoryenteritis.2. Question minimal gastric antral and duodenal wall inflammation. back

26. Peptic Ulcer Disease Correct – peptic ulcer disease may present with mouth sores, abdominal pain and weight loss. Fatigue would be less likely. back

27. Celiac Disease Correct – celiac disease may present with weight loss, fatigue and decreased appetite but less likely mouth sores back

28. GI Bleed Incorrect – at this time we have no evidence of anemia; there is no report of hematochezia or hematemesis back

29. Infection Incorrect – unlikely at this time; there is no report of fever or diarrhea back

30. Post Viral Gastroparesis Correct – can decrease gastric empty transit time and cause symptoms of early satiety, decreased appetite and weight loss; depending on the virus pre-ceding the diagnosis there may have been mouth sores (hand, foot and mouth disease) back

31. Ulcerative Colitis (UC) Yes & No – while UC is a form of IBD it is limited to the colon and most times should not be associated with oral aphthous ulcers back

32. Crohn’s Disease Correct – mouth sores can frequently be associated with inflammation anywhere in the gastrointestinal (GI) tract from the mouth to the anus Additionally, all other symptoms of weight loss, fatigue and early satiety can be explained by a diagnosis of Crohn’s Disease back

33. Lactose Intolerance Incorrect – while there may be a component of lactose intolerance; this is more likely to be secondary to a primary disease process such as Celiac Disease or Crohn’s Disease back

34. Oral Prednisone • Immunosuppressant - Prolonged use of corticosteroids may increase the incidence of secondary infection, mask acute infection (including fungal infections), prolong or exacerbate viral infections, or limit response to killed or inactivated vaccines. Exposure to chickenpox or measles should be avoided • Bridge to long term controller medication – prednisone to be used to quickly bring symptoms under control but wean off medication in 2-3 months while starting long term controller medication • Side effects: suicidal ideation and mood fluctuations, growth velocity and bone mineralization (osteoporosis), ocular disease, hepatic impairment, blood sugar fluctuations, GI bleed and/or myopathy • Taper as directed is extremely important due to adrenal suppression back

35. Mercaptopurine (6MP) • Immunomodulator/immunosuppressive – immune responses to infections may be impaired and the risk for infection is increased. Common signs of infection, such as fever and leukocytosis may not occur; lethargy and confusion may be more prominent signs of infection • Side effects – sun burn more easily, a rare type of cancer called hepatosplenic T-cell lymphoma (HSTCL) has happened with this drug, pancreatitis, lymphoma and hepatotoxicity back

36. Hepatitis B ImmunizationImproveCareNow (ICN) consensus Red Book recommendations: • “Vaccine recipients who do not develop a serum response (> or = 10 mIU/mL) after primary vaccine series should be re-immunized with an additional 3-dose series. People who remain negative 1-2 months after re-immunization series are unlikely to respond and therefore do not require additional vaccines.” back

37. DXA Dual-energy X-ray absorptiometry (DXA, previously DEXA) is a means of measuring bone mineral density (BMD). Two X-ray beams with different energy levels are aimed at the patient's bones. When soft tissue absorption is subtracted out, the BMD can be determined from the absorption of each beam by bone. back

38. Labs at time of endoscopy • Hepatitis B Surface Antibody • QuantiFERON TB Gold • TPMT Enzyme – for initiation of mercaptopurine • Urine Histoplasma Antigen – this is recommended prior to initiation of anti-TNF therapy as it can be reactivated following initiation of anti-TNF treatment and is one of the most common opportunistic infections in patients receiving these medications These labs were not completed prior to endoscopy as they are expensive and may be denied by insurance if IBD is not confirmed. back

39. Pancreatitis Correct – however less likely since pain is able to be ignored and there is no report of vomiting. There is value to obtaining a baseline lipase since pancreatitis can be a side effect of some IBD treatment. back