Ulcerative Colitis
Ulcerative Colitis: Excerpt from The 5-Minute Pediatric Consult
Jonathan Markowitz, MD
Ulcerative Colitis - BASICS
Ulcerative Colitis - description
Ulcerative colitis (UC) is a disease characterized by remitting and relapsing inflammation of the large intestine. UC and Crohn disease (CD) are the disorders that represent the idiopathic inflammatory bowel diseases (IBDs). The hallmark symptoms of UC are abdominal cramping, diarrhea, and bloody stools. There are multiple patterns of presentation in children. UC always affects the rectum, with contiguous involvement extending proximally, including up to the entire large intestine.
Ulcerative Colitis - epidemiology
- Yearly incidence is 2/100,000 in 10–19-year-olds
- 20% of patients with UC present before the age of 20
- Incidence peaks between 15 and 30 years of age.
- Total prevalence is 50–75 per 100,000.
Ulcerative Colitis - risk factors
Ulcerative Colitis - genetics
- HLA association: Bw52, DR2 (Japan); A2, Bw35, Bw40 (Ashkenazi Jews); A7, A11 (the Netherlands)
- Higher concordance in monozygotic than in dizygotic twins
Ulcerative Colitis - etiology
UC is thought to be a multifactorial disease. It likely results from a combination of individual genetic susceptibility and several environmental risk factors that may include:
- Hygiene/Exposure to bacteria/parasites/viruses/allergens
- Breast versus bottle feeding
- Specific bacteria that colonize the colon
- Dietary influences
- Smoking
Ulcerative Colitis - DIAGNOSIS
Home testing of stool for occult blood can alert patients and physicians to active disease.
Ulcerative Colitis - signs & symptoms
Ulcerative Colitis - history
A detailed history is important in making the diagnosis:
- Rectal bleeding (90%)
- Abdominal pain (90%)
- Diarrhea (50%)
- Weight loss (10%)
- Growth failure
- Recent travel (enteric infections)
- Antibiotic use (Clostridium difficile)
- Family history of IBD
- Appendectomy is protective against developing UC.
Ulcerative Colitis - physical exam
- Fever
- Evidence of weight loss or poor growth
- Signs of anemia
- Uveitis
- Mouth sores
- Arthritis
- Abdominal tenderness
- Abdominal distention
- Perianal/Rectal examination (UC should not be associated with perianal disease)
- Evidence of hepatobiliary disease
Ulcerative Colitis - tests
- CBC; anemia, iron deficiency
- Iron studies (iron deficiency)
- ESR; disease activity
- Electrolytes (hydration)
- Hepatic function panel (hepatobiliary disease)
- Perinuclear anti-neutrophil cytoplasmic antibody (pANCA; positive in 80% of UC patients, 20% of CD patients)
- Stool for blood, white cells (colitis)
- Stool cultures, C. difficile toxin A and B (infection)
Ulcerative Colitis - imaging
- Plain abdominal radiograph: This is important in diagnosing perforation, ileus, obstruction, and toxic megacolon. In toxic megacolon, the colon is dilated, and there are multiple air–fluid levels indicative of ileus. Serial x-rays are mandatory.
- Barium enema can demonstrate strictures and mucosal disease.
- An upper GI with small-bowel follow-through (UGI/SBFT) can demonstrate the entire small bowel to exclude small intestinal disease indicative of CD.
- MRI may have a role in differentiating transmural and mucosal inflammation, and is also useful for demonstrating perianal fistulae that would indicate CD rather than UC.
- Ultrasound may be useful for evaluating associated hepatobiliary disease.
- Radionuclide imaging can differentiate between CD (small and large bowel involvement) and UC (only large bowel involvement).
Ulcerative Colitis - diag proced-surgery
- Colonoscopy (with biopsies) is necessary to confirm the diagnosis of UC.
- Upper endoscopy may increase chances of detecting CD.
- Endoscopic retrograde cholangiopancreatography (ERCP) is useful in diagnosing primary sclerosing cholangitis (3% of UC patients).
- Video capsule endoscopy (VCE) is more sensitive than UGI/SBFT for diagnosing small bowel disease indicative of CD rather than UC.
- Pitfalls:
- The combination of positive pANCA and negative ASCA (anti-Saccharomyces cerevisiae antibody) has a reported sensitivity of 60–70% and a specificity of 95–97% for UC in adults. The sensitivity and specificity are poorer in pediatric patients.
- Inflammation of the small intestine demonstrated by colonoscopy, VCE, UGI/SBFT, or radionuclide imaging is suggestive of CD, not UC.
- Perianal disease (perianal skin tags, perianal fistulae, perianal abscess) is indicative of CD, not UC.
- Infectious colitis (especially C. difficile) can mimic the findings of UC. C. difficile infection must be evaluated with assays for both toxin A and toxin B, or up to 40% of infections can be missed.
- Toxic megacolon is a surgical emergency. The patient has a dilated colon with breakdown of its barrier to toxins entering the systemic circulation. Signs and symptoms include peritonitis, mental status changes, and fluid and electrolyte imbalance. Plain abdominal radiograph shows a segment or total colonic dilatation. Risk factors include 1st attack, pancolitis, concurrent use of opiates or anticholinergics, and recent barium enema or colonoscopy.
Ulcerative Colitis - pathological findings
- Inflammation and ulceration of colonic mucosa with acute inflammatory cells infiltrating the villi, lamina propria, and crypts, giving rise to crypt abscesses
- Inflammation is mucosal, but can infiltrate all layers of the bowel.
- Site of colon affected:
- Rectum (virtually 100%)
- Left side (50–60%)
- Pancolitis (10%)
- Small intestine should not be involved, but occasionally the terminal ileum can show some inflammation on radiologic or histologic examination. This is thought to be from refluxed colonic contents (backwash ileitis).
- Skip lesions are not seen in UC.
- Chronic gastritis may be present in patients with UC.
Ulcerative Colitis - differencial diagnosis
- CD
- Infectious colitis: Salmonella, Shigella, Campylobacter, Yersinia, Escherichia coli (enterohemorrhagic), Aeromonas, Amebiasis, C. difficile, cytomegalovirus
- STDs (herpes simplex, lymphogranuloma inguinale, Chlamydia)
- Trauma due to anal sex or sexual abuse
- Congenital Hirschsprung enterocolitis
- Bleeding juvenile polyps
- Milk protein allergy
- Eosinophilic colitis
- Autoimmune enteropathy
- Irritable bowel syndrome (IBS)
- Appendicitis
- Hemolytic–uremic syndrome
- Henoch–Schönlein purpura
Ulcerative Colitis - TREATMENT
Ulcerative Colitis - initial stabilization
Emergency care is indicated for fulminant disease. Initial evaluation should include abdominal examination, laboratory evaluation (CBC, ESR, electrolytes), and abdominal obstruction series (supine and upright abdominal films). Surgical consultation should be obtained in cases of suspected toxic megacolon.
Ulcerative Colitis - medication
- Mild disease can be treated with oral mesalamine, topical corticosteroid enema or foam, or mesalamine enema/suppositories.
- Moderate disease: Mesalamine, a short course of oral corticosteroid, low-residue diet
- Immunomodulators such as azathioprine and 6-mercaptopurine may help to maintain disease remission and minimize the need for recurrent courses of steroid. Methotrexate has fewer published data in UC, but also may have efficacy.
- Fulminant disease: Hospitalization, complete bowel rest with total parenteral nutrition, broad-spectrum antibiotics (IV ampicillin, gentamicin, and metronidazole), IV corticosteroids, serial abdominal radiographs, frequent examinations, stool chart (frequency, amount of blood, and volume of stool output); early surgical consult
- If treatment of acute symptoms with IV steroids fails, therapy with infliximab (usually given as a 5 mg/kg infusion) can be begun. A 2nd dose is usually given ~2 weeks after the initial infusion.
- Infliximab may also be used in cases of chronically active, but not fulminant, UC. The efficacy for this indication may be lower than for fulminant disease, however.
- IV cyclosporine infusion is an alternative to infliximab for treating fulminant colitis.
- Patients with fulminant disease who fail therapy with infliximab or cyclosporine should be referred for colectomy. Those with chronically active disease unresponsive to medication should also consider colectomy.
- Infliximab has recently been demonstrated to have efficacy in UC and may represent an alternative to cyclosporine for unresponsive disease.
- Therapy of toxic megacolon is aimed at preventing perforation with decompression of the bowel. Management includes complete bowel rest, discontinuation of anticholinergics and narcotics, not performing endoscopy or barium enema, and broad-spectrum antibiotics; frequent examinations are required. Close communication with surgical colleagues is crucial. In the absence of improvement after 24–72 hours, the patient requires urgent surgery.
- Methylprednisolone (IV): 1–2 mg/kg/d (equivalent to prednisone 60 mg maximum)
- Prednisone (PO): 1–2 mg/kg/d oral (up to maximum 60 mg/d)
- Mesalamine (PO): 40–60 mg/kg/d (maximum 4.8 g/d)
- Mesalamine (enema): 4 g at bedtime
- Mesalamine (suppository): 500 mg b.i.d.
- Hydrocortisone enema: 100 mg once a day to b.i.d.
- Hydrocortisone foam: 80 mg once a day to b.i.d.
- 6-mercaptopurine (6-MP) (PO): 1.0–1.5 mg/kg to start (keep [ANC] >500)
- Azathioprine (PO): 2.0 mg/kg (keep absolute neutrophyte count [ANC] >500)
- Infliximab (IV): 5 mg/kg 2 weeks apart, then q6–12wk as needed.
- Cyclosporine (IV): 4 mg/kg/d for 2 weeks (therapeutic levels vary depending on the technique used in the laboratory)
- Cyclosporine (oral): 6–8 mg/kg/d for 6–8 months
Ulcerative Colitis - surgery
- Urgently required for perforation, significant and persistent bleeding, toxic megacolon, and failure of medical treatment for fulminant colitis
- Can be electively performed for chronic incapacitating disease, growth failure, dysplastic changes in the colon, or longstanding disease (usually after 10 years)
- Ileoanal anastomosis and pouch construction is surgery of choice for most pediatric patients.
Ulcerative Colitis - FOLLOW UP
Outpatient follow-up with a pediatric gastroenterologist should be arranged. Important parameters to follow as an outpatient include abdominal symptoms, stool frequency/consistency, height/weight, hemoglobin, WBC count (for patients on immunosuppressives), ESR, albumin, bilirubin and liver enzymes, fecal occult blood testing, colonoscopic cancer screening (patients with longstanding disease). An index for monitoring disease in pediatric patients (the Pediatric Ulcerative Colitis Activity Index) is in the final stages of development.
Ulcerative Colitis - complications
- Bleeding
- Anemia
- Toxic megacolon
- Extraintestinal manifestations include hepatobiliary disease (3–5%), uveitis (up to 4%), arthritis affecting large joints (10%), spondylitis (6%), erythema nodosum (>5%), pyoderma gangrenosum (>1%), renal calculi (5%)
- Malignancy risk is 0.51% per year after a decade of onset of disease. The risk for adenocarcinoma of the colon in children who developed disease before 14 years of age is 40% by 40 years of age.
- Colonic stricture
Ulcerative Colitis - bibliography
- Baldassano RN, Piccoli DA. Inflammatory bowel disease in pediatric and adolescent patients. Gastroenterol Clin North Am. 1999;28(2):445–458.
- Becker JM. Surgical therapy for ulcerative colitis and Crohn’s disease. Gastroenterol Clin North Am. 1999;28(2):371-390, viii-ix.
- Eidelwein AP, Cuffari C, Abadom V, et al. Infliximab efficacy in pediatric ulcerative colitis. Inflamm Bowel Dis. 2005;11:213–218.
- Escher J, Taminiau J, Nieuwenhuis E, et al. Treatment of inflammatory bowel disease in childhood: Best available evidence. Inflamm Bowel Dis. 2003;9:34–58.
- Greifer MK, Markowitz JF. Update in the treatment of paediatric ulcerative colitis. Expert Opin Pharmacother. 2006;7:1907–1918.
- Gremse DA, Crissinger KD. Ulcerative colitis in children: Medical management. Paediatr Drugs. 2002;4:807–815.
- Hyams J, Markowitz J, Lerer T, et al. The natural history of corticosteroid therapy for ulcerative colitis in children. Clin Gastroenterol Hepatol. 2006;4:1118–1123.
- Jarnerot G, Hertervig E, Friis-Liby I, et al. Infliximab as rescue therapy in severe to moderately severe ulcerative colitis: A randomized, placebo-controlled study. Gastroenterology. 2005;128:1805–1811.
- Katz S. Update in medical therapy of ulcerative colitis: Newer concepts and therapies. J Clin Gastroenterol. 2005;39:557–569.
- Lewis JD, Deren JJ, Lichtenstein GR. Cancer risk in patients with inflammatory bowel disease. Gastroenterol Clin North Am. 1999;28(2):459–477.
- Michetti P, Peppercorn MA. Medical therapy of specific clinical presentations. Gastroenterol Clin North Am. 1999;28(2):353-370, viii.
- Papadakis KA, Targan SR. Current theories on the causes of inflammatory bowel disease. Gastroenterol Clin North Am. 1999;28(2):283–296.
- Rutgeerts P, Sandborn WJ, Feagan BG, et al. Infliximab for induction and maintenance therapy for ulcerative colitis. N Engl J Med. 2005;353:2462–2476.
- Stein RB, Hanauer SB. Medical therapy for inflammatory bowel disease. Gastroenterol Clin North Am. 1999;28(2):297–321.
Ulcerative Colitis - CODES
Ulcerative Colitis - icd9
556 Ulcerative colitis
Ulcerative Colitis - FAQ
- Q: Will my child have this disease forever?
- A: Some people will have only the initial attack and then be symptomfree, but usually an individual will have episodes of recurrences and remissions. Surgical removal of the colon represents a curative procedure, although some patients may develop inflammation in the pouch created out of the remaining bowel (pouchitis).
- Q: What is the cause of UC?
- A: Both genetic and environmental factors are important in the development of UC.
- Q: Where can I learn more about UC?
- A: The Children’s Digestive Health and Nutrition Foundation provides a Web site for children with IBD and their families (www.KidsIBD.org). The Crohn’s and Colitis Foundation of America (www.CCFA.org) is a nonprofit organization dedicated to the care and education of people with CD and UC.
- Q: What new therapies will be used in the near future?
- A: Biologic agents, a broad category of therapies that uses our recently improved knowledge of the immune system, represent a new way of treating IBD, with several new treatments likely to be released within the next few years.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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