Ulcerative Colitis
Ulcerative colitis is a chronic disease in which the large intestine becomes inflamed and ulcerated (pitted or eroded), leading to flare-ups (bouts or attacks) of bloody diarrhea, abdominal cramps, and fever.
Ulcerative colitis may start at any age but usually begins between the ages of 15 and 30. A small group of people have their first attack between the ages of 50 and 70.
Ulcerative colitis usually does not affect the full thickness of the wall of the large intestine and rarely affects the small intestine. The disease usually begins in the rectum or the sigmoid colon (the lower end of the large intestine) and eventually spreads along the partial or entire length of the large intestine. In some people, most of the large intestine is affected early on.
Ulcerative proctitis, which is confined to the rectum, is a very common and more benign form of ulcerative colitis.
The cause of ulcerative colitis is not known, but heredity and an overactive immune response in the intestine may be contributing factors. Cigarette smoking, which is detrimental in Crohn's disease, appears to actually decrease the risk of ulcerative colitis. However, smoking in order to reduce the risk of ulcerative colitis is ill-advised in light of the many health problems that smoking can cause.
Symptoms
The symptoms of ulcerative colitis occur in flare-ups. A flare-up may be sudden and severe, producing violent diarrhea, high fever, abdominal pain, and peritonitis (inflammation of the lining of the abdominal cavity). During such flare-ups, the person is profoundly ill. More often, a flare-up begins gradually, and the person has an urgency to have a bowel movement (defecate), mild cramps in the lower abdomen, and visible blood and mucus in the stool. A flare-up can last days or weeks and can recur at any time.
When the disease is limited to the rectum and the sigmoid colon, the stool may be normal or hard and dry; however, mucus containing large numbers of red and white blood cells is discharged from the rectum during or between bowel movements. General symptoms of illness, such as fever, are mild or absent.
If the disease extends farther up the large intestine, the stool is looser, and the person may have 10 to 20 bowel movements a day. Often, the person has severe abdominal cramps and distressing, painful rectal spasms that accompany the urge to defecate. There is no relief at night. The stool may be watery and contain pus, blood, and mucus. Frequently, the stool consists almost entirely of blood and pus. The person also may have a fever and a poor appetite and may lose weight.
Complications
Bleeding, the most common complication, often causes iron deficiency anemia. In nearly 10% of people with ulcerative colitis, a rapidly progressive first attack becomes very severe, with massive bleeding, perforation, or widespread infection.
Toxic colitis, a particularly severe complication, involves damage to the entire thickness of the intestinal wall. The damage causes ileus--a condition in which the normal contractile movements of the intestinal wall temporarily stop--so that the intestinal contents are not propelled along their way. Abdominal expansion (distention) develops. As toxic colitis worsens, the large intestine loses muscle tone, and within days--or even hours--it starts to expand. X-rays of the abdomen show gas inside the paralyzed sections of intestine.
Toxic megacolon occurs when the large intestine greatly expands (distends). The person is severely ill and may have a high fever. The person also has pain and tenderness in the abdomen and a high white blood cell count. If the intestine ruptures, the risk of death is great. However, of the people who receive prompt treatment before rupture occurs, fewer than 4% die.
Colon cancer occurs in as many as 1 of 100 people with ulcerative colitis each year in the later stages of their illness; 10 of 100 people with extensive ulcerative colitis develop colon cancer over their lifetime. The risk of colon cancer is highest when the entire large intestine is affected and the person has had ulcerative colitis for more than 8 years, regardless of how active the disease is. Colonoscopy (examination of the large intestine using a flexible viewing tube) every 1 to 2 years is advised for people who have had ulcerative colitis for at least 8 years. During colonoscopy, tissue samples are obtained throughout the large intestine for microscopic examination. Most people survive if the diagnosis of cancer is made during the cancer's early stages.
Other complications can occur, as in Crohn's disease. When ulcerative colitis causes a flare-up of gastrointestinal symptoms, the person also may experience inflammation of the joints (arthritis), inflammation of the whites of the eyes (episcleritis), inflamed skin nodules (erythema nodosum), and blue-red skin sores containing pus (pyoderma gangrenosum). When ulcerative colitis is not causing a flare-up of gastrointestinal symptoms, the person still may experience inflammation of the spine (ankylosing spondylitis), inflammation of the pelvic joints (sacroiliitis), and inflammation of the inside of the eye (uveitis).
Although people with ulcerative colitis commonly have minor liver dysfunction, only about 1 to 3% have symptoms of mild to severe liver disease. Severe liver disease can include inflammation of the liver (chronic active hepatitis); inflammation of the bile ducts (primary sclerosing cholangitis), which narrow and eventually close; and replacement of functional liver tissue with scar tissue (cirrhosis). Inflammation of the bile ducts may appear many years before any intestinal symptoms of ulcerative colitis; bile duct inflammation greatly increases the risk of cancer of the bile ducts and may even increase the risk of colon cancer.
Diagnosis
The person's symptoms and a stool examination help the doctor establish the diagnosis. Blood tests reveal anemia, increased numbers of white blood cells, a low level of the protein albumin, and an elevated erythrocyte sedimentation rate (ESR), which indicates active inflammation. A sigmoidoscopy (an examination of the sigmoid colon using a flexible viewing tube) confirms the diagnosis and permits a doctor to directly observe the severity of the inflammation. Even during symptom-free intervals, the intestine rarely appears entirely normal, and tissue samples removed for microscopic examination usually show chronic inflammation.
X-rays of the abdomen may indicate the severity and extent of the disease. Barium enema x-ray studies and colonoscopy are not usually done before treatment begins because they pose a risk of perforation when done during the active stages of the disease. At some point, however, the entire large intestine is usually evaluated by colonoscopy to determine the extent of the disease.
Prognosis and Treatment
Usually, ulcerative colitis is chronic, with repeated flare-ups and remissions. A rapidly progressive initial attack results in serious complications in about 10% of people. Complete recovery after a single attack may occur in another 10%. However, some people who have only a single attack may actually have ulcerations from an undetected infection rather than true ulcerative colitis.
People who have ulcerative proctitis (inflammation and ulceration that are confined to the rectum) have the best prognosis. Severe complications are unlikely; however, in about 10 to 30% of people, the disease eventually spreads to the large intestine (thus evolving into ulcerative colitis).
Treatment aims to control the inflammation, reduce symptoms, and replace any lost fluids and nutrients.
Dietary Restrictions: Iron supplements may offset anemia caused by ongoing blood loss in the stool. Raw fruits and vegetables should be avoided to reduce injury to the inflamed lining of the large intestine. A diet free of dairy products may decrease symptoms and is worth trying but need not be continued if no benefit is noted.
Antidiarrheal Drugs: Anticholinergic drugs or small doses of loperamide or diphenoxylate are taken for relatively mild diarrhea. For more intense diarrhea, higher doses of diphenoxylate or deodorized opium tincture, loperamide, or codeine may be needed. In severe cases, a doctor closely monitors the person taking these antidiarrheal drugs to avoid precipitating toxic megacolon.
Anti-inflammatory Drugs: Drugs such as sulfasalazine, olsalazine, mesalamine, and, most recently, balsalazide, are used to reduce the inflammation of ulcerative colitis and to prevent flare-ups of symptoms. These drugs usually are taken by mouth, but mesalamine can also be given as an enema or a suppository. Whether given by mouth or rectally, these drugs are at best moderately effective for treating mild or moderately active disease and for maintaining remission.
People with moderately severe disease who are not confined to bed usually take oral corticosteroids such as prednisone. Prednisone in fairly high doses frequently induces a dramatic remission. After prednisone controls the inflammation of ulcerative colitis, sulfasalazine, olsalazine, or mesalamine often is given to maintain the improvement. Gradually, the prednisone dosage is decreased, and ultimately, the prednisone is discontinued. Prolonged corticosteroid treatment almost invariably produces side effects. The new corticosteroid budesonide has fewer side effects than prednisone but may not be as effective. When mild or moderate ulcerative colitis is limited to the left side of the large intestine (descending colon) and the rectum, enemas with a corticosteroid or mesalamine may be given.
If the disease becomes severe, the person is hospitalized, and corticosteroids and fluids are given intravenously. People with heavy rectal bleeding may require blood transfusions.
Immunomodulating Drugs: Drugs such as azathioprine and mercaptopurine have been used to maintain remissions in people with ulcerative colitis who would otherwise need long-term corticosteroid therapy. These drugs inhibit the function of T cells, which are an important component of the immune system. However, these drugs are slow to act, and a benefit may not be seen for 2 to 4 months. They also have potentially serious side effects that require close monitoring by the doctor. Cyclosporine has been given to some people who are suffering severe flare-ups and have not responded to corticosteroid therapy. Many of these people respond initially to the cyclosporine, but some may still ultimately require surgery.
Surgery: Surgery may be necessary for unremitting chronic disease that would otherwise make the person an invalid or chronically dependent on high doses of corticosteroids. In rare cases, severe colitis-related problems outside the intestine, such as blue-red skin sores containing pus (pyoderma gangrenosum) or severe blood clotting in deep veins of the legs or arms, may make surgery necessary.
Surgery is performed on a nonemergency basis when cancer is diagnosed or precancerous changes (dysplasia) are identified in the large intestine. Surgery also may be performed because of a narrowing of the large intestine or growth retardation in children. Complete removal of the large intestine and rectum permanently cures ulcerative colitis. Living with a permanent ileostomy (a surgically created connection between the lowest portion of the small intestine and an opening in the abdominal wall) and an ileostomy bag has been the traditional price of this cure. However, various alternative procedures are available, the most common one being a procedure called ileo-anal anastomosis. In this procedure, the large intestine and most of the rectum are removed, and a small reservoir is created out of the small intestine and attached to the remaining rectum just above the anus. This procedure maintains continence, although some complications, such as inflammation of the reservoir, may occur.
For people with ulcerative proctitis, surgery is rarely needed, and life expectancy is normal. In some people, though, the symptoms may prove exceptionally resistant to treatment.
Toxic colitis is an emergency that may require surgery. As soon as a doctor detects it or suspects impending toxic megacolon, all antidiarrheal drugs are discontinued, the person is given nothing to eat, a tube is inserted through the nose and into the stomach or small intestine and attached to intermittent suction, and all fluids, nutrition, and drugs are given intravenously. The person is monitored closely for signs of peritonitis or a perforation. If these measures fail to improve the person's condition in 24 to 48 hours, emergency surgery is needed: All or most of the large intestine is removed.
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