$Unique_ID{BRK03879} $Pretitle{} $Title{Interstitial Cystitis} $Subject{Interstitial Cystitis Hunner's Ulcer or Syndrome Submucosal Cystitis Panmural fibrosis Submucosal Ulcer of the Bladder Elusive Ulcer IC Cystitis Colli Bladder cancer Endometriosis Radiation Cystitis } $Volume{} $Log{} Copyright (C) 1986, 1988, 1990, 1991 National Organization for Rare Disorders, Inc. 103: Interstitial Cystitis ** IMPORTANT ** It is possible that the main title of the article (Interstitial Cystitis) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Hunner's Ulcer or Syndrome Submucosal Cystitis Panmural fibrosis Submucosal Ulcer of the Bladder Elusive Ulcer IC Information on the following disorders can be found in the Related Disorders section of this report. Cystitis Colli Bladder cancer Endometriosis Radiation Cystitis 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. Interstitial cystitis is an ulcerative, slowly progressive, inflammatory disorder of the various layers of the wall of the urinary bladder. The patient experiences urinary frequency, small bladder capacity, and varying degrees of pain. It afflicts primarily middle aged women. Most patients respond to drug therapy, but surgery is required in some patients. Symptoms The urgent need to urinate often, including at night, and the ability to urinate only small amounts at a time are the primary characteristics of interstitial cystitis. Typically, there is also pain above the pubic area, which slowly disappears upon voiding. If urination must be delayed, the pain can be excruciating, and the urine may contain blood from cracks or fissures in the bladder wall. Patients are often tense and anxious. Symptoms gradually become more severe over the course of months or years. Painful sexual intercourse is also a common problem. The most common complications are infections following the use of medical instruments in examination or treatment procedures, and hydronephrosis. Hydronephrosis is the distention of parts of the kidney with urine that is backed up due to narrowing of the passage from the kidney to the bladder, or to reflux of urine from the bladder to the kidney. Prolonged hydronephrosis can damage the kidney. Cytoscopic examination of the bladder reveals thickened, inelastic walls, small star shaped ulcerations, and a decreased volume. With distention, bleeding spots and fissures in the mucosal lining often appear. Inflammation and fibrosis (scarring) of bladder wall, replacement of muscle by fibrous tissue, a thin and patchy mucosal layer, increased numbers of blood vessels, which however show degenerative changes, and infiltration of the wall with white blood cells associated with inflammatory reactions characterize the microscopic pathology of the bladder. Causes The idea that interstitial cystitis is an autoimmune disease of the connective tissue is gaining more and more support. Many patients have a history of allergies, but few report having had infections. Radiation therapy and treatment with drugs such as cyclophosphamide, used in cancer therapy and as an immunosuppressant, can also cause cystitis which resembles interstitial cystitis. Affected Population Interstitial Cystitis usually women over the age of 40 years, although about 10% of the patients are men. Younger women occasionally have the disorder. Many patients have a history of frequent allergies. According to one study, about 1 in 5600 women develop this disorder. It is estimated that 20,000 to 90,000 cases of IC have been diagnosed in the United States. Related Disorders Cystitis colli (cystanchenitis) is characterized by inflammation of the neck of the bladder, whereas Interstitial Cystitis involves inflammation of the walls of the bladder. Bladder cancer is marked by malignant growths on the bladder walls which may cause local destruction and spread or even recur after treatment. Endometriosis is characterized by the presence of endometrium tissue (which normally lines the uterus), found in abnormal locations. If the endometrium spreads to be bladder, symptoms may resemble those of Interstitial Cystitis. Radiation Cystitis is characterized by formation of cysts inside the bladder as a result of radiation treatments. Therapies: Standard In Interstitial Cystitis, one of the most common treatments designed to increase bladder capacity is repeated hydraulic distention of the bladder, with or without anesthesia. Lavage with a silver nitrate solution, or electrofulguration (electrocoagulation) may permit healing of ulcerations. Other substances which may be applied directly to the lesions by irrigation include dimethyl sulfoxide (DMSO), oxychlorosene sodium (e.g., clorpactin WCS- 90), and cortisone acetate. Glucocorticoids administered orally, and anticholinergic drugs such as propantheline bromide or oxybutinyn chloride may also relieve symptoms in some cases. Oral medications include anti- inflammatory drugs, antispasmodic drugs, antihistamines and muscle relaxants. If these measures all fail, the bladder may be enlarged, using tissue from the intestine as a "patch" (ceco- or ileo- cystoplasty). In the most severe cases, it may be necessary to remove the bladder and divert the urine to the small or large intestine. Therapies: Investigational One of the most common treatments for Interstitial Cystitis is designed to increase bladder capacity by repeated hydraulic distention of the bladder, usually under general anesthesia. One researcher used neodymium-YAG laser treatment on five patients with severe Interstitial Cystitis who failed to respond to conventional therapy. Cessation of severe bladder pain and frequency of urination within several days after therapy was noted in four of these patients. Bladder capacity was found to be increased overall, and complication rates from this modality of therapy were extremely low. The patients, who were followed for 3 to 15 months after treatment, have had no recurrent symptoms of severe disease, although some have had mild recurrent voiding symptoms. Long-term data on potential complications resulting from this kind of therapy is not yet available. Elmiron (sodium pentosan polysulphate), an orphan drug used experimentally in treatment of Interstitial Cystitis, is now being distributed in the United States by: Medical Market Specialties, Inc. P.O. Box 150 Boonton, NJ 07005 (201) 263-4243 This company is seeking 150 people with Interstitial Cystitis to participate in clinical trials. Physicians are asked to contact the company at the above address if they are interested in this experimental treatment. Anti-inflammatory drugs such as Benzydamine, antihistamines, and heparin sodium may be useful in some cases. A five year research program began in 1987 at the University of Pennsylvania to develop a comprehensive database on Interstitial (IC) patients and examine possible causes of the disorder. Research is also being focused on the GAG layer which acts as a protective lining in the bladder and, if defective, might permit the penetration of substances in the urine which could cause IC. Researchers are also exploring substances in the urine which might irritate the bladder wall. Possible hormonal, immunological, and infectious causes of IC are also being investigated. A research project at the Tufts New England Medical Center is exploring the role of mast cells (implicated in allergic reactions) and their possible relation to Interstitial Cystitis (IC). A new drug, Nalmefene, has received approval from the FDA to begin clinical trials at various sites throughout the United States. Trials should begin this summer. Nalmefene is manufactured by Cummins Pharmaceuticals, Inc. The FDA is testing Nifedipine, an orphan drug developed by Dr. Jonathan Fleischmann, for the treatment of Interstitial Cystitis. This disease entry is based upon medical information available through July 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 Interstitial Cystitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Interstitial Cystitis Association of America (East Coast) 18 Cedar Lane Ossining, NY 10562 (212) 979-6047 Interstitial Cystitis Association of America (West Coast) P.O. Box 151323 San Diego, CA 92175 National Kidney and Urologic Diseases Information Clearinghouse Box NKUDIC Bethesda, MD 20892 (301) 468-6345 References Smith, Donald R. General Urology, tenth edition. Chapter 28, pp 470-84. Lange Medical Publications, Los Altos, CA: 1981. The treatment of interstitial cystitis by cystolysis with observations on cystoplasty. A review after 7 years. Worth, P.H. Br J Urol 1980 Jun; 52(3):32. Successful treatment of interstitial cystitis with sodium pentosanpolysulfate. Parsons, C.L., et al. J Urol 1983 Jul; 130(1):51-3. Interstitial cystitis. Observations on diagnosis and on treatment with anti-inflammatory drugs, particularly benzydamine. Walsh, A. Eur Urol 1977; 3(4):216-7.