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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.