eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Ulcerative Colitis

Author: Jonathan E Markowitz, MD, Associate Professor of Clinical Pediatrics, University of South Carolina School of Medicine; Attending Pediatric Gastroenterologist, Associate Director of Pediatric Residency Program, Greenville Hospital System
Coauthor(s): Petar Mamula, MD, Assistant Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine; Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania; David A Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine; Liz D Dancel, MD, Intern, Department of Pediatrics, Greenville Hospital System University Medical Center
Contributor Information and Disclosures

Updated: Mar 27, 2008

Introduction

Background

Ulcerative colitis (UC) is a disease characterized by remitting and relapsing inflammation of the large intestine. UC and Crohn disease (CD) account for the disorders that represent the inflammatory bowel diseases (IBDs). The hallmark symptoms of UC include abdominal cramping, diarrhea, and bloody stools. Many patterns of presentation are possible in the pediatric age group. UC is generally considered to always affect the rectum, with contiguous involvement that can include the entire large intestine.

Pathophysiology

UC is characterized by accumulations of polymorphonuclear neutrophils in the crypts of the colon (crypt abscesses) with epithelial ulceration, edema, and hemorrhage. These changes may represent the final common pathway of a heterogeneous group of diseases relating to genetic and environmental factors.

The mucosal immune system of the large intestine is continuously exposed to a wide array of antigens from ingested food products and from the billions of bacteria that live there. When activated, the cells of the mucosal immune system release cytokines that recruit inflammatory cells to the tissue and perpetuate the inflammatory response. In patients with IBD, the inflammatory response to luminal antigens may be exaggerated over that seen in healthy individuals or they may not respond normally to down-regulation. The patient's genetic background likely determines when and how the inflammatory cells of the mucosal immune system react to the environment.

Frequency

United States

About 20% of patients with UC present before age 20 years. The incidence rate for UC in North Americans aged 10-19 years is approximately 2 cases per 100,000 persons. No simple Mendelian genetic mechanism explains the transmission of IBD, yet many familial occurrences are known in 15-20% of patients. Patients in whom disease is diagnosed before age 20 years appear to have an associated family history of the disease.

Race

Early studies tended to show a higher prevalence of UC in white Americans than in black Americans. Over time, this trend seems to be decreasing, with recent incidence rates nearly the same. Asian Americans appear to have low incidences. Several studies have shown that the incidence of IBD in white Americans is up to 4 times higher in Jewish people than among other racial or ethnic groups.

Sex

No sex predilection for UC is reported.

Age

• In most studies, incidence of UC peaks between adolescence and early adulthood (ie, in people aged 15-30 y).
• A smaller peak occurs in patients aged 60-80 years.
• UC occurs less frequently in children younger than 5 years than in others.

Clinical

History

Ulcerative colitis (UC) is a diffuse mucosal inflammation limited to the colon that affects the rectum and may extend proximally in a symmetric uninterrupted pattern to involve parts or all of the large intestine. Because UC is a mucosal disease limited to the colon, the most common presenting symptoms are rectal bleeding, diarrhea, and abdominal pain. Many patterns of presentation occur in the pediatric age group.

• Mild disease is observed in 50-60% of patients. This presentation involves insidious onset of diarrhea, later associated with hematochezia. No systemic findings of fever, weight loss, or hypoalbuminemia are observed. UC is typically confined to the distal colon and responds well to therapy.
• Moderate disease is observed in 30% of patients and is characterized by bloody diarrhea, cramps, urgency to defecate, and abdominal tenderness. Associated systemic findings, such as anorexia, weight loss, low-grade fever, and mild anemia, are present.
• Severe colitis occurs in approximately 10% of patients. This presentation involves more than 6 bloody stools per day, abdominal tenderness, fever, anemia, leukocytosis, and hypoalbuminemia. Patients with severe colitis may experience life-threatening complications, including severe hemorrhage, toxic megacolon, or intestinal perforation.
• Less than 5% of children with UC present with predominantly extraintestinal manifestations, such as growth failure, arthropathy, skin manifestations, or liver disease.
• Recently, a clinical scoring system known as the Pediatric Ulcerative Colitis Activity Index (PUCAI) has been developed and validated.¹  The PUCAI may be used more frequently to assess disease activity in clinical trials involving pediatric patients with UC.

Physical

The findings on physical examination in UC vary depending on the extent, duration, and severity of the disease. In addition to abdominal signs, many extraintestinal manifestations of UC may become evident during physical examination.

• Vital signs may indicate fever.
  • Tachycardia may represent anemia or hypovolemia.
  • Tachypnea may be present because of abdominal splinting or as a compensatory mechanism for acidosis in cases of severe dehydration.
• Comparison with growth charts may reveal delayed growth.
  • Cushingoid appearance is indicative of steroid use, usually over a long period. The patient may appear toxic in cases of fulminant disease.
  • Long-standing and severe disease may cause signs of malnutrition, such as muscle wasting.
• Abdominal examination findings are sometimes normal, but examination is likely to reveal abdominal tenderness.
  • Voluntary or involuntary guarding may be present.
  • Bowel sounds may be normal, hyperactive, or hypoactive.
  • Rushes or high-pitched tinkling may be found in cases of obstruction.
  • Rebound tenderness indicates severe disease and possible perforation.
  • A palpable mass may indicate obstruction or megacolon.
  • An enlarged spleen may be indicative of portal hypertension from associated autoimmune hepatitis or primary sclerosing cholangitis.
  • Long-standing disease can be associated with colonic stricture and evidence for bowel obstruction.
• Skin examination may reveal pallor in cases of anemia, decreased skin turgor in cases of dehydration, and jaundice, caput medusae, or spider angiomata when associated liver involvement is present. Erythema nodosum may be evident on extensor surfaces (but more common in Crohn disease [CD] than UC); pyoderma gangrenosum affects approximately 1% of patients with UC.
• Patients with episcleritis can present with a painful erythematous eye.
  • Cataracts may occur in patients with significant steroid history.
  • Scleral icterus may be indicative of liver disease.
• Joint pain (arthralgia) is a common finding in inflammatory bowel disease (IBD), although swollen or red joints (arthritis) occur less frequently. The large peripheral joints, such as the knees, ankles, wrists, and elbows, are most commonly involved, but any joint can be involved. Approximately 1% of patients with UC develop ankylosing spondylitis; most of these patients test positive for human leukocyte antigen (HLA) type B27.
• Unlike what is seen in some patients with CD, perianal examination in patients with UC should not reveal any evidence of fistulae or abscesses; however, chronic diarrhea may lead to perianal erythema, fissuring, or hemorrhoids.
• Sexual development may be delayed in patients with UC, but this finding is more common in patients with CD than in UC.

Causes

UC is thought to be a multifactorial disease.

• Data from genetic epidemiologic studies strongly support the role of genetic factors in UC. Concordance is higher among monozygotic twins than among dizygotic twins; however, the lack of perfect concordance in monozygotic twins suggests other factors in the etiology of UC as well.
• Several environmental factors have been implicated in the pathogenesis of IBD.
  • Smoking influences the course of IBD (positively in UC, negatively in CD).
  • Certain infections have been implicated in IBD (eg, measles, atypical mycobacteria infection).
  • Patients with IBD have been shown to have different colonizing bacteria than people without IBD.

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