Crohn's disease

Definition

Crohn's disease is a type of inflammatory bowel disease (IBD), resulting in swelling and dysfunction of the intestinal tract.

Description

Crohn's disease involves swelling, redness, and loss of function of the intestine, especially the small intestine. There is evidence that this inflammation is caused by a misfire of the immune system, which attacks the body itself instead of attacking foreign invaders, such as viruses or bacteria. The inflammation of Crohn's disease most commonly occurs in the last part of the ileum (a section of the small intestine), and often includes the large intestine (the colon). However, inflammation may also occur in other areas of the gastrointestinal tract, including the mouth, esophagus, or stomach. Crohn's disease differs from ulcerative colitis, the other major type of IBD, in two important ways:

  • The inflammation of Crohn's disease may be discontinuous, meaning that areas of involvement in the intestine may be separated by normal, unaffected segments of intestine. The affected areas are called "regional enteritis," while the normal areas are called "skip areas."
  • The inflammation of Crohn's disease affects all the layers of the intestinal wall, while ulcerative colitis affects only the lining of the intestine.

Also, ulcerative colitis does not usually involve the small intestine; in rare cases it involves the terminal ileum (so-called "backwash" ileitis).

In addition to inflammation, Crohn's disease causes ulcerations, or irritated pits, in the intestinal wall. These pits occur because the inflammation has made areas of tissue shed away.

While Crohn's disease and ulcerative colitis are similar, they are also very different. Although it can be difficult to determine whether a patient has Crohn's disease or ulcerative colitis, it is important to make every effort to distinguish between these two diseases. Because the long-term complications of the diseases are different, treatment will depend on careful diagnosis of the specific IBD present.

Crohn's disease may be diagnosed at any age, although most diagnoses are made between the ages of 15–35. About 20–40 people out of 10,000 suffer from this disorder, with men and women having an equal chance of being stricken. Caucasians are more frequently affected than other racial groups, and people of Jewish origin appear three to six times more likely to suffer from IBD. IBD runs in families; an IBD patient has a 20% chance of having other relatives who are fellow sufferers.

Crohn's disease is a chronic disorder. While the symptoms can be improved, there is no known cure for the underlying disease.

Causes & symptoms

The cause of Crohn's disease is unknown. No infectious agent (virus, bacteria, or fungi) has been identified as the etiologic agent. Still, some researchers have theorized that some type of infection may have originally been responsible for triggering the immune system, resulting

A double-contrast barium x ray showing the colon of a patient with Crohn's disease. (Custom Medical Stock Photo. Reproduced by permission.)

in the continuing and out-of-control cycle of inflammation that occurs in Crohn's disease. Other evidence for a disorder of the immune system includes the high incidence of other immune disorders that may occur along with Crohn's disease.

The first symptoms of Crohn's disease may include diarrhea, fever, abdominal pain, inability to eat, weight loss, and fatigue. Some patients experience severe pain that mimics appendicitis. It is rare, however, for patients to notice blood in their bowel movements. Because Crohn's disease severely limits the ability of the affected intestine to absorb the nutrients from food, a patient with Crohn's disease can have signs of malnutrition, depending on the amount of intestine affected and the duration of the disease.

The combination of severe inflammation, ulceration, and scarring that occurs in Crohn's disease can result in serious complications, including obstruction, abscess formation, and fistula formation.

An obstruction is a blockage in the intestine. This obstruction prevents the intestinal contents from passing beyond the point of the blockage. The intestinal contents "back up," resulting in constipation, vomiting, and intense pain. Although rare in Crohn's disease (because of the increased thickness of the intestinal wall due to swelling and scarring), a severe bowel obstruction can result in an intestinal wall perforation (a hole in the intestine). Such a hole in the intestinal wall would allow the intestinal contents, usually containing bacteria, to enter the abdomen. This complication could result in a severe, life-threatening infection.

Abcess formation is the development of a walledoff pocket of infection. A patient with an abscess will have bouts of fever, increased abdominal pain, and may have a lump or mass that can be felt through the wall of the abdomen.

Fistula formation is the formation of abnormal channels between tissues. These channels may connect one area of the intestine to another neighboring section of intestine. Fistulas may join an area of the intestine to the vagina or bladder, or they may drain an area of the intestine through the skin. Abscesses and fistulas commonly affect the area around the anus and rectum (the very last portions of the colon allowing waste to leave the body). These abnormal connections allow the bacteria that normally live in the intestine to enter other areas of the body, causing potentially serious infections.

Patients suffering from Crohn's disease also have a significant chance of experiencing other disorders. Some of these may relate specifically to the intestinal disease, and others appear to have some relationship to the imbalanced immune system. The faulty absorption state of the bowel can result in gallstones and kidney stones. Inflamed areas in the abdomen may press on the tube that drains urine from the kidney to the bladder (the ureter). Ureter compression can make urine back up into the kidney, enlarge the ureter and kidney, and can potentially lead to kidney damage. Patients with Crohn's disease also frequently suffer from:

  • arthritis (inflammation of the joints)
  • spondylitis (inflammation of the vertebrae, the bones of the spine)
  • ulcers of the mouth and skin
  • painful, red bumps on the skin
  • inflammation of several eye areas
  • inflammation of the liver, gallbladder, and/or the channels (ducts) that carry bile between and within the liver, gallbladder, and intestine

The chance of developing cancer of the intestine is greater than normal among patients with Crohn's disease, although this chance is not as high as among those patients with ulcerative colitis.

Diagnosis

Diagnosis is first suspected based upon a patient's symptoms. Blood tests may reveal an increase in certain types of white blood cells, an indication that some type of inflammation or infection is occurring in the body. The blood tests may also reveal anemia and other signs of malnutrition due to malabsorption (low blood protein; variations in the amount of calcium, potassium, and magnesium present in the blood; changes in certain markers of liver function). Stool samples may be examined to make sure that no infectious agent is causing the diarrhea, and to see if the waste contains blood.

A colonoscopy may be performed to view the interior of the colon. During colonoscopy, a doctor passes a flexible tube with a tiny, fiber-optic camera device (an endoscope) through the rectum and into the colon. The doctor can then carefully examine the lining of the intestine for signs of inflammation and ulceration that might suggest Crohn's disease. A tissue sample (a biopsy) of the intestine can also be taken through the endoscope to examine under a microscope for evidence of Crohn's disease.

Both an upper and lower GI (gastrointestinal) x ray series can be helpful in determining how much of the intestine is involved in the disease. In the upper GI (also called a small bowel series), the patient drinks a chalky solution called barium, which acts as a contrast agent to illuminate the gastrointestinal tract on x-ray film. After the barium is ingested, x rays are taken at specific time intervals as the barium passes through the stomach and into and through the small intestine. The lower GI series provides an x-ray study of the large intestine. The patient is given an enema containing barium, and in some cases, air is also pumped into the rectum to provide a clearer view of the large intestine. This is called a double-contrast barium enema.

Treatment

Crohn's disease is a chronic, often progressive, illness. A correct diagnosis and appropriate treatment with anti-inflammatory medications is critical to controlling the disease.

Some Crohn's patients find that certain foods are hard to digest, including milk, large quantities of fiber, and spicy foods. Dietary adjustments are usually necessary to minimize pain, diarrhea, and other symptoms.

Acupuncture and guided imagery may be useful tools in treating any pain associated with Crohn's disease. Acupuncture involves the placement of thin needles into the skin at targeted locations on the body known as acupoints in order to harmonize the energy flow within the human body. To treat chronic pain, such as that involved with Crohn's disease, an acupuncturist will frequently place the acupuncture needles along what is known as the large intestine meridian.

Guided imagery involves creating a visual mental image of one's pain in one's mind. Once the pain can be visualized, the patient can adjust the image to make it more pleasing, and thus, more manageable.

Several herbal remedies are also available to lessen pain symptoms and promote relaxation and healing. These include peppermint oil, slippery elm (Ulmus rubra), marsh mallow (Althaea oficinalis), and Chinese herbs. However, Crohn's patients should consult with their healthcare professional before taking them. Depending on the preparation and the type of herb, these remedies may aggravate the digestive tract or interact with any prescription drugs that are being taken to control the inflammation of Crohn's disease.

Allopathic treatment

Treatments for Crohn's disease try to reduce the underlying inflammation, the resulting malabsorption/malnutrition, the uncomfortable symptoms of crampy abdominal pain and diarrhea, and any possible complications (obstruction, abscesses, and fistulas).

Inflammation can be treated with a drug called sulfasalazine. Sulfasalazine is made up of two parts. One part is related to the sulfa antibiotics; the other part is a form of the anti-inflammatory chemical, salicylic acid. Sulfasalazine is not well-absorbed from the intestine, so it stays mostly within the intestine, where it is broken down into its components. It is believed that the salicylic acid component actively treats Crohn's disease by fighting inflammation. Some patients do not respond to sulfasalazine, particularly those with more severe disease. These patients require steroid medications (such as prednisone). Steroids, however, must be used carefully to avoid the complications of these drugs, including increased risk of infection and weakening of bones (osteoporosis)

In 2001, the Food and Drug Administration (FDA) approved use of budesonide capsules for mild and moderate cases of Crohn's disease involving the small and large intestines. Although a steroid, the makeup of budesonide allows the drug to release into the intestines, where it can be mostly metabolized. As a result, less of the drug enters the patient's system, meaning fewer undesirable side effects. Some potent immunosuppressive drugs that interfere with the products of the immune system and hopefully decrease inflammation may be used for those patients who do not improve on steroids.

Serious cases of malabsorption/malnutrition may need to be treated by providing nutritional supplements. These supplements must be in a form that can be absorbed from the damaged, inflamed intestine. When patients are suffering from an obstruction, or during periods of time when symptoms of the disease are at their worst, they may need to drink specially formulated, high-calorie liquid supplements. Those patients who are severely ill may need to receive their nutrition through a needle inserted intravenously.

A number of medications are available to help decrease the cramping and pain associated with Crohn's disease. These include loperamide, tincture of opium, and codeine. Some fiber preparations (methylcellulose or psyllium) may be helpful, although some patients do not tolerate them well.

The first step in treating an obstruction involves general attempts to decrease inflammation with sulfasalazine, steroids, or immunosuppressive drugs. A patient with a severe obstruction will have to stop taking all food and drink by mouth, allowing the bowel to "rest." Abscesses and other infections will require antibiotics. Surgery may be required to repair an obstruction that does not resolve on its own, to remove an abscess, or to repair a fistula. Such surgery may involve the removal of a section of the small intestine. In extremely severe cases of Crohn's disease of the colon that do not respond to treatment, a patient may need to have the entire large intestine removed (an operation called a colectomy). In this case, a piece of the remaining small intestine is pulled through an opening in the abdomen. This bit of intestine is fashioned surgically to allow a special bag to be placed over it. This bag catches the body's waste, which no longer can be passed through the large intestine and out of the anus. This opening, which will remain in place for life, is called an ileostomy. However, as an alternative to ileostomy, small intestines are now often shaped into substitute rectal pouches, and the patient may not always need the ileostomy.

Expected results

Crohn's disease is a lifelong illness. The severity of the disease can vary, and a patient can experience periods of time when the disease is not active and he or she is symptom free. However, the complications and risks of Crohn's disease tend to increase over time. Well over 60% of all patients with Crohn's disease will require surgery, and about half of these patients will require more than one operation over time. About 5–10% of all Crohn's patients will die of their disease, primarily due to massive infection.

Prevention

Crohn's disease is a chronic, lifelong disorder. However, a study published in the New England Journal of Medicine in June 2000 reported that methotrexate (a chemotherapy drug) was found to prevent relapse episodes in a clinical trial of Crohn's patients. The study also found that human growth hormone was useful in reducing symptoms of the disease.

Resources

BOOKS

Glickman, Robert. "Inflammatory Bowel Disease: Ulcerative Colitis and Crohn's Disease." In Harrison's Principles of Internal Medicine. Anthony S. Fauci et al., eds. New York: McGraw-Hill, 1998.

Long, James W. The Essential Guide to Chronic Illness. New York: HarperPerennial, 1997.

Saibil, Fred. Crohn's Disease and Ulcerative Colitis. Buffalo, NY: Firefly Books, 1997.

PERIODICALS

Peppercorn, Mark A., and Susannah K. Gordon. "Making Sense of a Mystery Ailment: Inflammatory Bowel disease."Harvard Health Letter 22, no. 2 (December 1996): 4+.

Sachar, David. "Maintenance Strategies in Crohn's Disease." Hospital Practice 31, no. 1 (January 15, 1996): 99+.

Karpa, Kelly Dowhower. "Crohn's disease patients find new relief from old drug."Drug Topics 145, no. 21 (November 5, 2001): 16;.

ORGANIZATIONS

Crohn's & Colitis Foundation of America, Inc. 386 Park Avenue South, 17th Floor, New York, NY 10016-8804. (800) 932-2423.

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