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Chapter 66

Ulcerative Colitis

Dorin T. Colibaseanu and David W. Larson

Key Points

1 Ulcerative colitis is a form of inflammatory bowel disease (IBD) largely limited to the mucosa and

submucosa of the colon and rectum. This process is continuous, beginning at the rectum, and

extending proximally for a variable distance.

2 The acute indications for surgical intervention are fulminant disease, toxic megacolon, and bleeding.

Failures of medical management, dysplasia, or cancer are indications for elective surgery.

3 The intestinal manifestations of ulcerative colitis (UC) are cured by removal of the colon and

rectum.

4 Current surgical options depend on the acuity of presentation of the disease; minimally invasive

methods should be employed when clinically indicated and technically feasible.

5 Proctocolectomy with ileal pouch-anal anastomosis (IPAA) provides patients with a continent

reservoir; a Brooke ileostomy is available for those patients who do not qualify for a pouch

construction.

6 Although perioperative and late complications after ileal pouch-anal reconstruction are not

uncommon, the vast majority of patients enjoy a high quality of life after surgery.

INTRODUCTION

1 Ulcerative colitis (UC) and Crohn disease (CD) affect more than 1.4 million people in the United

States. Together these two diseases have many common characteristics, yet they are two distinct

entities.1 UC is a nonspecific and idiopathic inflammatory bowel disease (IBD) of unknown etiology that

primarily affects the mucosa and submucosa of the colon and rectum. It is characterized by recurring

episodes of inflammation. While medical treatment remains the mainstay of UC management, it does

not represent a cure.

EPIDEMIOLOGY

Determining the exact incidence of UC is difficult because mild cases might never be reported, and the

disease itself is uncommon. In North America, the reported incidence varies between 2.2 and 19.2 cases

per 100,000 person-years.1–5 Global incidence and prevalence of UC tends to be the highest along

northern latitudes, and lower in Asia and the Middle East.5–8 Over time, the incidence of IBD has

increased globally, especially the last 20 years.9,10

Etiology and Risk Factors

Development of UC is likely due to the interaction of multiple genes, and environmental factors. It is

more common in Jewish and Caucasian populations, and infrequent in black and Hispanic

populations.11,12

Genetics

Both environmental and genetic risk factors have been implicated in the development of UC; however,

the exact pathogenesis has not been fully elucidated. Up to 20% of the patients with IBD are part of

families with other afflicted members. It is recognized that there is a non-Mendelian genetic

predisposition to developing UC. Monozygotic twin concordance rate for UC varies between 10% and

19%, and it increases over time.13–15 The offspring of parents with UC can have a risk of developing UC

as high as 33%.16 Genetic concordance in CD is higher than in UC, and more than 100 loci have been

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associated with increased risk for developing IBD (Table 66-1).17–21 Major histocompatibility complex

HLA-DR2 is associated with UC, and there are at least 15 distinct loci that are associated with risk of

developing UC.22–25

Environmental Factors

There remains a bimodal distribution of UC, which typically develops between 15 and 40 years of age

and then in a smaller subgroup later in life between 50 and 80 years (Fig. 66-1).26 UC appears to be

slightly more common in men.27,28

Table 66-1 Genes Commonly Implicated in Ulcerative Colitis and Crohn Disease

Figure 66-1. Age-specific incidence of UC in Midwestern US population. (Adapted from Loftus CG, Loftus EV Jr, Harmsen WS, et

al. Update on the incidence and prevalence of Crohn’s disease and ulcerative colitis in Olmsted County, Minnesota, 1940–2000.

Inflamm Bowel Dis 2007;13:254–261.)

Diet rich in saturated fats, and refined sugar has been associated with higher incidence of UC.

Physical activity however, is not strongly associated to incidence of UC.29–31 Smoking appears to have a

protective effect for UC; conversely former smokers have an increased risk of UC compared to those

who never smoked.32–34 The apparent protective effect of smoking extends to some of the

extraintestinal manifestations of UC, in particular primary sclerosing cholangitis (PSC) and occurrence

of pouchitis.35,36 Interestingly, an appendectomy also has a protective effect from developing UC,

though the mechanism is essentially unknown. Large, retrospective studies have shown a protective

effect of 55% to 70%,37–41 especially if the findings at the operation were consistent with appendicitis or

lymphadenitis.39 The mechanistic theories proposed for this interesting finding include the notion that

as the body’s immune system responds to the inflammatory process of appendicitis, it adapts such that

the inappropriate inflammatory process that defines UC never occurs. The environmental risks for

development of CD and UC have some similarities but the diseases are in essence distinct entities (Table

66-2).

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CLINICAL FEATURES

UC symptoms parallel the degree of the disease. Typically, UC affects the rectum, extending proximally

in a continuous fashion, and can involve the entire colon. Most commonly the distal colon is involved

for various length, however, approximately 20% of the time, patients present with pancolitis.42 The

classic symptoms of UC are diarrhea, and rectal bleeding. Additional symptoms include tenesmus,

urgency, and colicky abdominal pain.43 Persistent pain is uncommon, and often can represent severe

disease with inflammation extending to the serosa.44 The sensation of constipation and incomplete

evacuation can occur if there is severe rectosigmoid inflammation and spasm. As the disease progresses,

systemic symptoms of fever, anorexia, weight loss, and malaise ensue. Severity of disease can be

calculated using the Mayo Severity Index Calculator (Table 66-3).45 Other calculators exist.

Extraintestinal Disease

Hepatobiliary

Approximately 3% to 5% of UC patients develop PSC. Generally, the patients are asymptomatic, though

as the disease progresses liver enzyme elevation, pruritus, fatigue, chills, and right upper quadrant pain

can develop. The risk of acquiring PSC is independent of colonic UC activity, and the risk persists after

proctocolectomy. In addition, PSC is an independent risk factor for developing dysplasia/cancer in

patients with UC.46

Ophthalmologic

Greater than 3% of patients with UC can develop iritis, episcleritis, or uveitis. Episcleritis severity can

parallel GI symptoms, but uveitis does not match GI disease activity. Though uveitis is less common, the

disease is more severe, and if not promptly treated can result in blindness. Topical steroids and

infliximab have successfully been used in treating UC uveitis.47

Table 66-2 Clinicopathologic Features of Ulcerative Colitis vs. Crohn’s

Table 66-3 The Mayo Severity Index

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Cutaneous

Manifestations of UC can include erythema nodosum and pyoderma gangrenosum (Fig. 66-2). Erythema

nodosum generally follows the disease progress of the GI symptoms, occurring in up to 4% of patients

with UC.48 These violet subcutaneous nodules can be treated with topical steroids, though most

commonly, systemic treatment of GI symptoms will also help with erythema nodosum. While pyoderma

gangrenosum occurs less frequently than erythema nodosum, its treatment is more difficult as the

disease course of pyoderma gangrenosum does not always parallel UC activity. Infliximab has shown

some promise in treating pyoderma gangrenosum.49

Musculoskeletal

These conditions are responsible for the most frequent extraintestinal complaints. Sacroileitis and

ankylosing spondylitis are associated with UC flairs, and remissions. Carriers of HLA-B27 are especially

prone to ankylosing spondylitis. Successful treatment of sacroiliatis with NSAIDs does not preclude

progression of spondylitis. Methotrexate has also been successfully used, and there are increasing data

of successful use of anti-TNF agents.50

DIAGNOSIS

Physical examination is often unrevealing, though generalized pain is often an indicator of severe

disease. A triad of tachycardia, fever, and leukocytosis are indicators for systemic toxicity and should

alert the clinician of impending need of surgical intervention. Vague discomfort, and perianal irritation

are common findings due to bowel movement frequency.

Imaging

Conventional x-rays can be useful in the rare cases of obstruction and/or perforation. The use of

contrast studies allow for the identification of signs of severe inflammation with loss of haustral

markings, although they have largely been replaced by more modern imaging modalities. Figure 66-3

shows distended colon with loss of haustral markings, concerning for toxic megacolon.

Abdominal computed tomography with oral and/or rectal contrast and CT enterography protocols are

the modern imaging techniques of choice and provide additional information regarding the severity of

disease. The presence of fistulas, phlegmon, mass, or abscess is an indication of a disease process other

than UC as these findings may in fact represent other diagnoses such as Crohn, diverticulitis, and cancer.

MRI has the advantage of limiting ionizing radiation exposure, benefiting the young especially, and

those that potentially may require multiple imaging studies over the years. Additionally, MRI has the

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