Ulcerative Colitis: Introduction

Introduction

Figure 1. Location of the colon in the body.
Inflammatory bowel disease encompasses two idiopathic, chronic, inflammatory diseases: Crohn's disease and ulcerative colitis. Crohn's disease and ulcerative colitis are disorders of unknown cause involving genetic and immunological influence on the gastrointestinal tract's ability to distinguish foreign from self-antigens. They share many overlapping epidemiological, clinical and therapeutic characteristics. In some patients, it is not possible to distinguish which form of inflammatory bowel disease is present (Figure 2).

Figure 2. Inflammatory bowel disease subsets.

There are, however, important pathological and clinical differences that distinguish these inflammatory disease processes. Clinically, Crohn’s disease tends to present more frequently with abdominal pain and perianal disease, whereas ulcerative colitis is more often characterized by gastrointestinal bleeding.

Cobblestoning mucosa and aphthous or linear ulcers characterize the endoscopic appearance of Crohn’s disease. Ulcerative colitis presents with diffuse continuous involvement of the mucosa. Radiographic studies of patients with Crohn’s disease characteristically show fistulas, asymmetry and ileal involvement. In contrast, radiographic studies of patients with ulcerative colitis show continuous disease without fistulizing or ileal disease (Figure 3).

Figure 3. Anatomic distribution of Crohn’s disease and ulcerative colitis.

Pathologically, Crohn's disease features mucosal discontinuity, transmural involvement and granulomas. In contrast, ulcerative colitis does not. Crypt abscesses and granulomas are present only in Crohn's disease. Figure 4 compares the appearance of the colon, the histology, and endoscopic views of normal, Crohn’s disease, and ulcerative colitis patients.

Figure 4. Comparison of colonic mucosa in normal, Crohn’s and ulcerative colitis patients; (top), gross; (center), histological; (bottom), endoscopic appearance.

Ulcerative colitis (UC) is an idiopathic inflammatory bowel disease that occurs more often in industrialized countries. This disease affects both men and women similarly. The disease may be acute and chronic with unpredictable relapses and remissions. Major advances have been made in many aspects of inflammatory bowel disease, including new information on the molecular basis of the disease, epidemiological considerations, immunology and genetics. The clinical and scientific understanding of ulcerative colitis has been greatly expanded far beyond our earlier knowledge.

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General Info

What is Ulcerative Colitis?

Ulcerative colitis is an idiopathic inflammatory bowel disease that affects the colonic mucosa and is clinically characterized by diarrhea, abdominal pain and hematochezia. The extent of disease is variable and may involve only the rectum (ulcerative proctitis), the left side of the colon to the splenic flexure, or the entire colon (pancolitis). The severity of the disease may also be quite variable histologically, ranging from minimal to florid ulceration and dysplasia. Carcinoma may develop. The typical histological (microscopic) lesion of ulcerative colitis is the crypt abscess, in which the epithelium of the crypt breaks down and the lumen fills with polymorphonuclear cells. The lamina propria is infiltrated with leukocytes. As the crypts are destroyed, normal mucosal architecture is lost and resultant scarring shortens and can narrow the colon.

Figure 5. Histology of ulcerative colitis

Systemic and Extra-Colonic Manifestations

Arthritic complications may occur in as many as 26% of patients with ulcerative colitis. Spondolylitis occurs in 3% of these patients. The arthritic symptoms may appear before the inflammatory bowel disease and do not necessarily follow the course of the intestinal disease. Twelve to 23% of patients with ulcerative colitis have peripheral arthritis, which affects large, weight-bearing joints such as knees or ankles. Arthritis signs and symptoms usually accompany exacerbations of ulcerative colitis.

Nineteen percent of patients with ulcerative pancolitis experience dermatological changes. Erythemia nodosum and pyoderma gangrenosum are commonly associated with this disease. Other dermatological sequelae include dermatitis, erythematous rash, psoriasis, carcinoma, urticaria, pityriasis, lupus erythematosus, vitiligo and ecchymosis.

Ocular manifestations of ulcerative colitis occur in 5% of patients with extensive disease or with Crohn's disease, and may include anterior uveitis, episcleritis and keratoconjunctivitis. Symptoms of these complications include headache, photophobia, blurred vision, burning and increased secretions from the eyes (Figure 6).

Figure 6. Extracolonic manifestations of ulcerative colitis.

In most situations, extraintestinal manifestations respond to standard medical therapy. On rare occasions, a total proctocolectomy may be necessary to control severe extraintestinal manifestations of this disease.

Classification

The extent of colonic mucosal involvement and severity of disease correlate with the clinical manifestations of ulcerative colitis. Approximately one-third of all patients with ulcerative colitis have involvement limited to the rectum (the distal 15 cm of the large intestine) or ulcerative proctitis. Ulcerative proctitis is endoscopically characterized by edema, erythema and loss of vascular markings. Granularity, friability, and frank ulceration are also seen in more severe disease.

Figure 7. Extent of bowel involvement in different degrees of ulcerative colitis.

Distal or left-sided colitis is found in patients in whom the inflammatory process extends from the rectum 40 cm. Disease activity does not extend beyond the splenic flexure, and there is evidence of chronic inflammation and chronic architectural distortion.

Pancolitis involves the portion of the colon beyond splenic flexure. It is characterized by hematochezia and diarrhea, and may be accompanied by abdominal pain and cramps, fever, and/or weight loss with persistent inflammation. Normal haustral markings disappear with generalized shortening and tubularization of the colon. In severe disease, the mucosa may be described as nodular with pseudopolyps, a reticular pattern, and discrete ulcer craters.

Incidence

The incidence of ulcerative colitis has remained fairly constant in those areas for which data are available for a number of years. Ulcerative colitis has been reported between 1.0 and 15.0 cases per 100,000. In general, the rates are highest in the Scandinavian countries, Great Britain and North America. The disease is uncommon in Asia, Africa and South America, although good data are generally lacking from underdeveloped countries where the rates seem to be low. Prevalence is higher among Jewish people born in Europe and the United States (Ashkenazi Jews) than among those born in Asia and Africa. The literature reports a slightly higher incidence of ulcerative colitis in females than males. It is most likely to occur in early adulthood, but disease presentation can occur in the fifth or sixth decade, and occasionally in the seventh or eighth decade. Diet, breast-feeding, oral contraceptives, and the cessation of cigarette smoking have been implicated as risk factors for ulcerative colitis. Studies indicate a decreased risk of ulcerative colitis for current smokers, however, former smokers are at increased risk of developing the disease.

Symptoms

The predominant symptom in ulcerative colitis is diarrhea, which can be associated with frank blood in the stool. The patient has frequent bowel movements, which may be small in volume, as a result of irritability of the inflamed rectum (proctitis). Other symptoms include abdominal or rectal pain, fever and weight loss. Although diarrhea is the dominant complaint in patients with ulcerative colitis, some patients may complain of constipation and rectal spasm.

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