$Unique_ID{BRK03617} $Pretitle{} $Title{Colitis, Ulcerative} $Subject{Colitis, Ulcerative Idiopathic, Non-Specific Ulcerative Colitis Idiopathic Proctocolitis Chronic, Non-Specific Ulcerative Colitis} $Volume{} $Log{} Copyright (C) 1986, 1990, 1991 National Organization for Rare Disorders, Inc. 87: Colitis, Ulcerative ** IMPORTANT ** It is possible that the main title of the article (Ulcerative Colitis) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Idiopathic, Non-Specific Ulcerative Colitis Idiopathic Proctocolitis Chronic, Non-Specific Ulcerative Colitis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Ulcerative Colitis is a non-specific inflammatory disease of the bowel characterized by chronic ulceration. The chief characteristic of this disorder is bloody diarrhea. This disease of unknown cause generally begins in the rectosigmoid area. It may involve only the left side of the colon or may eventually extend to involve the entire bowel. However, in some cases it may attack most of the large bowel simultaneously. The disease is usually chronic, with repeated periods of exacerbation and remission. Ulcerative colitis is an acute nonspecific inflammation of the colon characterized by multiple, irregular superficial ulcerations. Thickening of the wall of the colon with scar tissue and polyp like structures are the result of prolonged inflammatory reactions. Symptoms The symptoms of ulcerative colitis may first include malaise, weakness and fatigue. A vague abdominal discomfort may be associated with a slight change in the frequency and consistency of stools. Later there may be pain along with cramping in the abdomen, and urgency (tenesmus). Anorexia, loss of weight, and abdominal distention may also be present. Ulcerative colitis generally manifests itself by means of attacks of bloody diarrhea which may vary in intensity and duration, followed by intervals when the patient is fairly asymptomatic. There is an increased urgency to defecate, mild cramps in the lower abdomen, and the stools may contain mucus and blood. However, in some cases the onset of the attack may be explosive with acute symptoms. In this instance, episodes of diarrhea may be sudden and violent. Patients may develop high fever, signs of peritonitis (inflammation of the membrane lining the abdominopelvic walls) and a profound toxemic state may exist. Causes The exact cause of ulcerative colitis is unknown. The disorder may be related to immunological agents, or may be caused by an unknown environmental agent. There is a documented familial tendency. A study of the incidence of Ulcerative Colitis and Crohn's Disease conducted in Denmark (published in 1991) indicates that relatives of people with either of these inflammatory bowel diseases has a 10-fold increased risk of developing the disease. This study suggests that the cause of both disorders may be genetic. Psychological factors may also have an effect on this disorder, but are not considered a cause. Affected Population Ulcerative colitis may begin at any age, but its peak incidence is between the ages of 20 and 25 years. There is also a perceptible increase in frequency in the fifth and sixth decades of life. The disease is more prevalent among Jews, but all ethnic groups appear to be at risk. Therapies: Standard The treatment for ulcerative colitis depends upon the severity of the disease. Mild forms of the disease may be managed with nonspecific supportive measures. These include adequate physical and emotional relaxation. The patients may follow a normal diet refraining only from the roughage in raw fruits and vegetables. Approximately one third of patients find milk an irritant and it should also be eliminated. Anti-diarrheal agents may also be necessary. Sulfasalazine may be used in mild to moderate forms of the disease. In order to reduce gastrointestinal side effects of the drug (nausea, indigestion, and anorexia), it should be taken with meals and the dosage increased gradually by the patient's physician. In cases of either mild or moderate severity, hydrocortisone enemas may occasionally be effective in achieving remission especially if the disease is located in the rectum or left colon. If the disease is severe, corticosteroid therapy may be indicated. Intensive therapy with prednisone frequently induces remission. Sulfasalazine may be given to the patient in conjunction with prednisone. After improvement, it may be possible to gradually taper the dosage of the corticosteroid and ultimately withdraw it. Chronic fecal blood loss may require iron in order to prevent anemia. The more severe attacks of ulcerative colitis may require hospitalization and parenteral corticosteroid (e.g., Hydrocortisone IV) or ACTH therapy. When remission has been obtained, oral prednisone therapy may be substituted, then gradually reduced after continued improvement. Immunosuppressive drugs such as azathioprine and 6-mercaptopurine have also been used to treat some patients with this disorder. Occasionally surgery may be necessary (colectomy and ileostomy), and offers the only permanent cure. Newer surgeries which offer continence and/or preservation of the anal sphincter are now available. The prognosis of ulcerative colitis is uncertain. While in most cases the disease is chronic with repeated exacerbations and remissions, complete recovery has occurred after a single attack in about ten percent of patients. The disease may be more severe when the onset occurs after sixty years of age. Possible complications of the disease may include arthritis, inflammation of the tissues surrounding the bile ducts known as pericholangitis, or inflammation of the vertebrae known as ankylosing spondylitis. Further complications may include abcesses in the rectum or colon, fistulas or intestinal perforations, and skin and eye disorders. Carcinoma of the colon may develop, but the risk is higher in those patients with universal colitis of more than ten years duration. The FDA recently approved the drug Dipentum (olsalazine sodium) for the treatment of Ulcerative Colitis patients. Dipentum is not a sulfa-related drug and therefore does not have sulfa-related side effects. The drug is manufactured by Pharmcia Inc., Piscataway, NJ. Therapies: Investigational George D. Ferry, M.D. has been awarded a grant from the Office of Orphan Products Development, Food and Drug Administration, for his research on using Olsalazine to treat childhood Ulcerative Colitis. For more information, please contact: George D. Ferry, M.D. Baylor College of Medicine Gastroenterology Research Unit, Section of Gastroenterology Department of Pediatrics 1709 Dryden, Suite 1103 Houston, TX 77030-2403 (713) 791-3201 Studies are being conducted in the use of Sandoglobulin as a treatment for Ulcerative Colitis. Further investigation is needed to determine it's safety and effectiveness. Dr. Warren L. Beekan of the University of Vermont has received a New Grant Award for 1990 from the Office of Orphan Products Development for controlled trials using 4-ASA in the treatment of Ulcerative Colitis. The orphan drug Pamisyl (P-D) is being tested for treatment of mild to moderate Ulcerative Colitis in patients intolerant of sulfasalazine. The drug is being tested by Warren Beekan, M.D., University of Vermont, Burlington, VT. The FDA has approved the following drugs for testing as treatments for Ulcerative Colitis patients. The orphan drug Altracin (bacitracin) is being tested for treatment of antibiotic-associated pseudomembraneous enterocolitis caused by toxins A and B elaborated by clostridium difficile. The drug is manufactured by A.L. Laboratories, Ft. Lee, NJ. The orphan drug short-chain fatty acid solution is being tested for treatment of the active phase of Ulcerative Colitis with involvement restricted to the left side of the colon. The treatment is being tested by Richard Breuer, M.D., Evanston, IL. This disease entry is based upon medical information available through March 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Ulcerative Colitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Foundation of Ileitis and Colitis, Inc. 444 Park Avenue South New York, NY 10016 (212) 685-3440 National Digestive Diseases Information Clearinghouse Box NDIC Bethesda, MD 20892 (301) 468-2162 United Ostomy Association, Inc. 36 Executive Park, Suite 120 Irvine, CA 97214 (714) 660-8624 References THE MERCK MANUAL 15th ed.: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 801. CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 753-60, 787-8. FAMILIAL OCCURRENCE OF INFLAMMATORY BOWEL DISEASE, M. Orhold, MD, N Eng J Med, (January 10, 1991, issue 324 (2)). Pp. 84-88.