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- $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.
-
-