Interstitial Cystitis (IC)/Painful Bladder Syndrome (PBS)

• IC/PBS is an inflammatory disease of the bladder that can cause ulceration and bleeding of the bladder's lining and can lead to scarring and stiffening of the bladder.
• The symptoms of IC/PBS are
  • pelvic or bladder pain,
  • frequent urination, and
  • urinary urgency.
• IC/PBS has a variable course, meaning that symptoms can appear and disappear over time. Moreover, the intensity of symptoms varies among individuals and even within the same individual over time.
• The cause of IC/PBS is unknown, but abnormalities in the leakiness or structure of the lining of the bladder may play a role in the development of IC/PBS.
• Health care professionals make the diagnosis of IC/PBS based on the typical symptoms and the elimination of other conditions that may be responsible for the symptoms.
• Treatment for IC/PBS most commonly utilizes heparinoid drugs to help restore integrity of the bladder lining along with other oral medications. Bladder distension and intravesical drug therapy are other treatments that may provide relief in IC/PBS.

The urinary system consists of the kidneys, ureters, bladder, and urethra. The kidneys, a pair of purplish-brown organs, are located below the ribs toward the middle of the back. The kidneys remove water and waste from the blood in the form of urine, keeping a stable balance of salts and other substances in the blood. The kidneys also produce erythropoietin, a hormone that stimulates the formation of red blood cells. Narrow tubes called ureters carry urine from the kidneys to the bladder, a triangle-shaped, muscular chamber in the lower abdomen. Like a balloon, the bladder's muscular, elastic walls relax and expand to store urine and contract and flatten when urine empties through the urethra. The typical adult bladder can store about 1 ½ cups of urine.

Adults urinate about 1½ quarts of urine each day. The amount of urine varies depending on the fluids and foods a person consumes. The volume formed at night is about half that formed during the day.

Normal urine contains fluids, salts (for example, sodium and potassium) and waste products, but it is free of bacteria, viruses, and fungi. The wall of the bladder is isolated from urine and toxic substances by a coating on the inside lining of the bladder that discourages bacteria from attaching and growing on the bladder wall.

Picture of the urinary system

Interstitial cystitis (IC), also referred to as painful bladder syndrome (PBS), is a condition that causes bladder pain, bladder pressure, chronic urinary urgency (feeling the need to urinate immediately), and frequency (frequent urination). The symptoms of this condition vary among individuals from mild to severe and may even vary with time in the same individual. The term cystitis refers to any inflammation of the bladder. In contrast to bacterial cystitis that results from an infection in the bladder, a type of urinary tract infection (UTI), medical researchers haven't identified an infectious organism in people with interstitial cystitis.

Estimates of the number of people affected by IC/PBS vary widely and are dependent upon the criteria used for diagnosis. Many experts believe that about 3.3 million women in the U.S. (over age 18) may have IC/PBS, as well as 1.6 million men.

Despite a lack of consistency about the diagnosis of IC/PBS, studies agree that the majority of those affected are women. While individuals of any age may develop IC/PBS, including children, the average age of onset is around 40. Physicians do not consider IC/PBS a hereditary disorder, but multiple cases have occurred among some families, prompting ongoing investigation of the possible role of hereditary factors in the development of IC/PBS.

Observations from cystoscopy (visual examination of the inside of the bladder via a camera) have found that two patterns exist for IC, ulcerative and nonulcerative, depending upon the presence or absence of ulcerations or breaks in the bladder lining. Star-shaped ulcerations in the bladder wall are Hunner's ulcers. These appear in less than 10% of cases of IC/PBS in the U.S.

Over time, interstitial cystitis can cause physical damage to the bladder wall. Scarring and stiffening of the bladder wall may occur because of the chronic inflammation, leading to a decrease in bladder capacity. Glomerulations (areas of pinpoint bleeding) and small areas of bleeding may appear on the bladder wall.

No one knows what causes IC/PBS, but doctors believe that it is a real physical problem and not a result, symptom, or sign of an emotional problem.

One possible cause of IC/PBS is the disruption of the lining layer of the bladder (known as epithelium) that causes it to become leaky, allowing toxic substances in urine to irritate the bladder wall.

Other possible theories about the cause of IC/PBS include an abnormality of the immune response, such as an autoimmune reaction, the presence of an unidentified infection, or increased nervous system activation in the nerves to the bladder. None of these theories has been conclusively proven to cause IC/PBS.

Because physicians don't understand the cause of IC/PBS, there are no definite risk factors for developing the condition. However, women are more likely than men to develop IC/PBS.

Some associations with other medical conditions appear with IC/PBS. Women with IC/PBS are more likely to have had frequent urinary tract infections (UTIs) and to have had previous gynecologic surgery than women without IC/PBS. Certain chronic illnesses occur more frequently in people with IC/PBS than in the general population. Examples of these associated illnesses are inflammatory bowel disease, systemic lupus erythematosus, irritable bowel syndrome (IBS), vulvodynia (chronic discomfort in the vulvar area), allergies, endometriosis, and fibromyalgia. While medical researchers have described each of these conditions in at least some studies to be more common in people with IC/PBS than in the general population, there is no evidence that any of these conditions is the cause of IC/PBS.

The symptoms of IC/PBS vary greatly from one person to another but have some similarities to those of a urinary tract infection. They include

• decreased bladder capacity resulting in frequent urination of smaller amounts of urine day and night, referred to as urinary frequency;
• a strong, urgent need to urinate when the need to urinate arises, referred to as urinary urgency;
• bladder pain, bladder pressure, and feelings of pressure, pain, and tenderness around the bladder, pelvis, and perineum (the area between the anus and vagina or anus and scrotum) which may increase as the bladder fills and decrease as it empties;
• painful sexual intercourse (dyspareunia); and
• discomfort or pain in the penis and scrotum.

In most women, symptoms usually worsen around the time of their periods. As with many other illnesses, stress also may intensify the symptoms, but it does not cause them. The symptoms usually have a slow onset, and urinary frequency is the most common early symptom. As IC/PBS progresses over a few years, cycles of pain (flare-ups or flares) and remissions occur. Pain may be mild or so severe as to be debilitating. Symptoms can vary from day to day.

Urologists are physicians that specialize in diagnosis and treatment of diseases of the urinary tract, including interstitial cystitis. Some gynecologists also may treat patients with interstitial cystitis.

Because the symptoms of IC/PBS are similar to those of other disorders of the urinary system and because there is no definitive test to identify IC/PBS, doctors must exclude other conditions before making a diagnosis of IC/PBS. Among the disorders to be excluded are urinary tract or vaginal infections, bladder cancer, bladder inflammation or infection caused by radiation to the abdomen, eosinophilic and tuberculous cystitis, kidney stones, endometriosis, neurological disorders, sexually transmitted diseases, urinary tract infection with small numbers of bacteria, and, in men, chronic bacterial and nonbacterial prostatitis.

Medical tests that help identify other conditions include a urinalysis, urine culture, cystoscopy, and biopsy of the bladder wall and, in men, laboratory examination of prostatic secretions.

Physical examination

Physical examination of an individual with IC/PBS may reveal tenderness of the bladder either when pushing on the abdomen over the bladder (just above the pubic bone) or during the pelvic examination in women. No specific physical findings are associated with IC/PBS.

Laboratory tests

Urinalysis and urine culture: These tests can detect and identify the most common bacteria in the urine that may be causing symptoms. A health care professional obtains a urine sample either by catheterization or by the "clean catch" method. For a clean catch, the patient washes the genital area before collecting a sample of urine "midstream" in a sterile container. White and red blood cells and bacteria in the urine suggest an infection of the urinary tract that physicians can treat with antibiotics. If urine is sterile for weeks or months while symptoms persist, a doctor may consider a diagnosis of IC/PBS.

Culture of prostatic secretions: In men, the doctor can obtain a sample of prostatic fluid. This fluid is examined under the microscope for signs of an infection such as red and white blood cells and can be cultured for bacteria. Antibiotics treat prostatic infections.

Potassium sensitivity test: Doctors use a test known as the intravesical potassium sensitivity test (PST) in the evaluation of IC/PBS. In the PST test, health care professionals instill two different solutions (sterile water and a solution of potassium) separately into the bladder. The patient rates the severity of pain and/or urgency that occur when a health care professional instills each solution. People with normal bladder linings cannot tell the difference between the two solutions, while those with IBC/PBS and other conditions that affect the leakiness of the bladder wall will experience more pain when a health care provider injects the potassium solution into the bladder.

Cystoscopy under anesthesia with bladder distension: During cystoscopy, the doctor uses a cystoscope -- an instrument made of a hollow tube about the diameter of a drinking straw with several lenses and a source of light - to look inside the bladder and urethra. The doctor will also distend or stretch the bladder to its capacity by filling it with a liquid or gas. Because bladder distension is painful in IC/PBS patients, before the doctor inserts the cystoscope through the urethra into the bladder, the patient must be given either regional or general anesthesia. Cystoscopy with distension of the bladder with fluid can detect inflammation (visually or with biopsies), a thick and stiff bladder wall, and Hunner's ulcers. After a health care professional drains fluid from the bladder, small red spots, called glomerulations, that represent enlarged blood vessels and pinpoint areas of bleeding can be seen in the bladder's lining.

The doctor also may determine a patient's bladder capacity -- the maximum amount of liquid or gas the bladder can hold under anesthesia. (Without anesthesia, either pain or a severe urge to urinate limits capacity.) Most people without IC/PBS have normal or large maximum bladder capacities under anesthesia. A small bladder capacity (due to scarring) under anesthesia helps to support the diagnosis of IC/PBS.

Cystoscopy has its limitations. Ulcers generally do not appear in mild or early cases of IC/PBS, and glomerulations have been observed in normal individuals without symptoms of IC/PBS. Due to these limitations, physicians recommend cystoscopy only to exclude other possible causes of symptoms and not as the definitive diagnostic test for IC/PBS.

It is important to note that the distension often performed with cystoscopy may lead to relief of symptoms in some patients with IC/PBS, which generally lasts from several weeks to months following the procedure.

Biopsy: A biopsy is a microscopic examination of a small sample of tissue. A physician may remove samples of the bladder and urethra during cystoscopy and examine them with a microscope later. A biopsy helps to exclude bladder cancer. It also may confirm the presence of mast cells or inflammation of the bladder wall that are consistent with a diagnosis of IC/PBS. Nevertheless, there is nothing on the biopsy that can make an absolute diagnosis of IC/PBS.

Oral medications

Oral medications are one treatment option for IC/PBS. These include low doses of antidepressants of the tricyclic group such as amitriptyline (Elavil). This is not due to a belief that IC/PBS is a psychological condition; rather, tricyclic antidepressants can help reduce the hyperactivation of nerves within the bladder wall. The antiseizure medication gabapentin (Neurontin, Gabarone) also treats nerve-related pain and has sometimes been used to treat the pain of IC/PBS. Physicians may prescribe oral antihistamines such as loratadine (Claritin) to help reduce allergic symptoms that may be worsening the patient's IC/PBS.

For patients who may not respond well to amitriptyline, another type of oral medication that can be used is the heparinoid (heparin-like) drug pentosan polysulfate sodium (PPS; brand name Elmiron). PPS is chemically similar to the substance that lines the bladder, and physicians believe that PPS assists in the protection of the inner lining cells of the bladder. It may take months before symptoms improve after treatment with this medication.

For those individuals who are unable to sleep well because of the discomfort of IC/PBS, antihistamines such as hydroxyzine can facilitate sleep.

People sometimes use aspirin and ibuprofen (Advil) as a first line of defense against mild discomfort. However, they may make symptoms worse in some patients. Over-the-counter forms of phenazopyridine hydrochloride (Azo-Standard, Prodium, and Uristat) may provide some relief from urinary pain, urgency, frequency, and burning. Higher doses of the drug are available by prescription as Prodium and phenazopyridine (Pyridium).

Bladder distension

As mentioned previously, because some patients have noted an improvement in symptoms after bladder distension done to diagnose IC/PBS, sometimes health care professionals use bladder distension (termed hydrodistension) for therapy of IC/PBS. Hydrodistension helps reduce symptoms in many people with interstitial cystitis. (Studies have shown an improvement in 40%-80% of patients.) When it is effective, the relief of symptoms persists for several weeks to months after the procedure. Physicians usually perform it under anesthesia or heavy sedation because of discomfort, and some patients have a temporary worsening of symptoms following the procedure.

Bladder instillation (intravesical therapy)

This procedure may also be called a bladder wash or bath. During a bladder instillation, the bladder is filled with a solution that is held for varying periods, from a few seconds to 15 minutes, before being drained through a narrow tube called a catheter.

In severe cases of IC/PBS, health care professionals administer intravesical solutions along with oral PPS to provide relief until the oral PPS has had time to take effect.

Drugs used for bladder instillations include dimethyl sulfoxide (DMSO, RIMSO-50), heparin, sodium bicarbonate, PPS, and hydrocortisone (a steroid).

Other therapies for interstitial cystitis

In severe cases of IC/PBS that do not respond well to oral medications or to bladder distension or instillation, other therapies may be attempted. A procedure known as sacral neuromodulation is effective in controlling symptoms in some people with IC/PBS. The term "neuromodulation" refers to an alteration of the nervous system. In sacral neuromodulation, a device is implanted that allows for stimulation of the nerves in the sacral (lower back) area. Sacral neuromodulation works by inhibiting the hyperactive signals from the sensory nerves within the bladder wall.

Transcutaneous electrical nerve stimulation (TENS) is a form of neuromodulation that does not involve surgical placement of wires or an impulse generator. With TENS, mild electric pulses enter the body for minutes to hours two or more times a day through wires placed on the surface of the lower back or the suprapubic region, between the navel and the pubic hair, or through special devices inserted into the vagina in women or into the rectum in men. Physicians believe that the electric pulses may increase blood flow to the bladder, strengthen pelvic muscles that help control the bladder, and trigger the release of hormones that block pain. TENS is generally more effective in reducing pain than in reducing urinary frequency.

Surgical procedures that may rarely be performed to treat severe IC/PBS include peripheral denervation (disrupting the nerves to and from the bladder wall), bladder augmentation to increase bladder capacity, and cystectomy (bladder removal) with diversion, or re-routing, of urine flow into an artificial bladder formed from a loop of intestine.

There are a number of home remedies and self-care techniques that many people have found to be of benefit in controlling the symptoms of interstitial cystitis/painful bladder syndrome. These include limiting the consumption of certain foods, smoking cessation, exercise, and bladder training.

Diet

There is no scientific evidence linking diet to IC/PBS, but doctors and patients believe that certain foods, including alcohol, spices, chocolate, and caffeinated and citrus beverages, may contribute to bladder irritation and inflammation. Foods containing acid, for example, carbonated beverages, tomatoes, vitamin C, citrus fruits and beverages, vinegar, cranberries, strawberries, grapes, guava, mango, and pineapple are believed to aggravate IC/PBS. Other foods that may increase symptoms because they contain the natural chemical tyramine include wine, beer, cheese, nuts, yogurt, bananas, soy sauce, chicken livers, raisins, sour cream, avocados, canned figs, corned beef, fava beans, brewers' yeast, and chocolate.

Some people with IC/PBS also have noticed a worsening of symptoms after eating or drinking products containing artificial sweeteners. They may try eliminating such products from their diet and, if there is a reduction of symptoms, they can reintroduce them one at a time to determine which product seems to be aggravating their symptoms.

Smoking

Many people with IC/PBS feel that smoking worsens their symptoms. (Because smoking is the major known cause of bladder cancer, one of the best things a smoker can do for the bladder is to quit smoking.)

Exercise

Many IC/PBS patients feel that regular exercise helps relieve symptoms and, in some cases, hastens remission.

Bladder training

People who have found some relief from pain may then be able to reduce frequency using bladder training techniques. Methods vary, but the patient decides to urinate at designated times and uses relaxation techniques and distractions to help keep to the schedule. Gradually, the patient lengthens the time between urinations. A diary usually is helpful in keeping track of progress.

IC/PBS is a chronic condition characterized by periods of relapse or flare-ups and remission. Doctors do not fully understand why the symptoms worsen at particular times or disappear and then reappear months or years later. Symptoms may be mild or severe and may vary in intensity even in the same individual over time. There is no cure for IC/PBS, and treatments aim to reduce the severity of symptoms.

There has been no treatment shown to be effective in slowing the progression of the disease or in preventing recurrences.

Because doctors do not understand the cause, there are no known strategies for preventing IC/PBS. However, some self-care measures may help reduce the severity of symptoms (see section on home remedies).

Interstitial Cystitis Association of America
110 North Washington Street, Suite 340
Rockville, MD 20850
Phone: 1-800-HELP-ICA (435-7422) or 301-610-5300
Fax: 301-610-5308
Email: [email protected]
Internet: http://www.ichelp.org

American Urogynecologic Society
2025 M Street NW., Suite 800
Washington, DC 20036
Phone: 202-367-1167
Fax: 202-367-2167
Email: [email protected]
Internet: http://www.augs.org

European Society for the Study of Interstitial Cystitis
http://www.essic.eu