What Is Interstitial Cystitis?
- Inflammation is a protective reaction of the body tissue to irritation, injury, or infection. Inflammation of the bladder is called cystitis.
- When the inflammation is caused by bacterial infection, it is referred to as bacterial cystitis or just cystitis.
- Interstitial cystitis (IC) is a condition that causes pain and inflammation in the bladder when no infection is found. (Other causes of noninfectious inflammation of the bladder are also possible.)
- Inflammation of the bladder causes urinary frequency (frequent need to urinate), urgency (urgent need to urinate), pelvic pain, painful urination, incontinence, and nocturia (frequent need to urinate at night).
- Long-term inflammation of the bladder in people with IC can lead to scarring and stiffening of the bladder wall, which causes a decrease in the bladder capacity.
- Pinpoint areas of bleeding, called glomerulations or large ulcers, may occur in the lining of the bladder wall.
- IC is believed to be a syndrome initially presenting with mild symptoms and progressing to severe urgency and pelvic pain.
- According to the National Institutes of Health (NIH), IC is more common in women. It is believed that many patients have early forms of IC with a delayed diagnosis. The average age of onset of IC is 40 years.
What Causes Interstitial Cystitis?
Although many theories have been put forward, the cause of IC is unknown. The theories for the cause of IC include the following:
- Autoimmune: An autoimmune response is a physical response in which cells and antibodies of a person's body are directed against that person's own tissues. An autoimmune response to a bladder infection destroys the lining of the bladder wall. An unexplained association of IC has been found to exist with other autoimmune diseases such as inflammatory bowel disease, systemic lupus erythematosus, scleroderma, Sjogren syndrome, fibromyalgia, and atopic allergy. IC has a very high association with disorders of the bowel such as inflammatory bowel disease.
- Hereditary: Studies of mothers, daughters, and twins who have IC suggest a hereditary risk factor. However, no gene has yet been implicated as a cause of IC.
- Mast cell abnormalities: In some people with IC, special white blood cells called mast cells (associated with inflammation) are found in the bladder lining. Mast cells release histamine and other chemicals that cause inflammation of the bladder.
- Defect in bladder epithelium: The bladder has a specialized natural lining called the epithelium. The epithelium is protected from toxins in the urine by a layer of protein called glycosaminoglycan. In people with IC, this protective layer breaks down, allowing toxins to irritate the bladder wall and cause inflammation of the bladder.
- Neurogenic: The nerves that carry bladder sensations are inflamed, so pain is caused by events that are not normally painful (such as filling of the bladder).
- Infectious: Although no causative infective agent has been found in the urine of people with IC, an unidentified infectious agent may be the cause.
Probably, different processes occur in different groups of people with IC. It is also likely that different processes may affect each other, for example, a defect in the bladder epithelium may start the inflammation and stimulate mast cells to release histamine.
What Are the Signs and Symptoms of Interstitial Cystitis?
The symptoms of IC are similar to those of a urinary tract infection. They vary from person to person. Most people have some of the following signs and symptoms:
- Frequency: People with IC need to urinate more often than normal. A person with good health urinates a maximum of seven times a day and does not have to get up at night to urinate. A person with IC urinates frequently, both during the day and night. In early or very mild cases, frequency is sometimes the only symptom.
- Urgency: As frequency becomes more severe, it leads to urgency. Urgency may also be accompanied by pain, pressure, or spasms. Some people with IC feel a constant urge to urinate that never goes away, even right after urinating.
- Pain: People with IC may have bladder pain that gets worse as the bladder fills. Some people feel pain in other areas besides the bladder. The pain may be felt in the lower abdomen, lower back, urethra, or pelvic or perineal area. Men may feel pain in the scrotum, testicles, or penis. Women may experience pain in the vulva or vagina. The pain may be continuous or intermittent.
- Sexual difficulties: Women may have pain during intercourse, and men may have a painful orgasm.
- Sleep difficulties
- Depression
- Urinary incontinence (leakage)
Symptoms of some people with IC become worse after consumption of certain foods or drinks. They include tomatoes, spices, alcohol, chocolate, caffeinated and citrus beverages, and high-acid foods. Many people also find that symptoms become worse if they have stress (either physical or mental stress). In women, symptoms may vary with the menstrual cycle; symptoms often get worse during periods.
How Do Medical Professionals Diagnose Interstitial Cystitis?
IC is often diagnosed after other conditions are ruled out because there is no test specifically for IC. On average, people with IC experience symptoms for four years before the condition is diagnosed.
Because the symptoms of IC are similar to those of other disorders of the urinary system, the first step is to rule out other diseases before considering a diagnosis of IC. Other possible causes for the symptoms include the following:
- Urinary tract infections
- Bladder cancer
- Tuberculous cystitis
- Radiation cystitis
- Sexually transmitted diseases
- Kidney stones
- Vaginal infections
- Endometriosis
- Prostatitis
- Overactive bladder (OAB)
- Neurogenic bladder (bladder symptoms caused by a neurologic disease)
Tests that help to rule out these other conditions include the following:
- Urine culture: This test can be used to identify the organisms that cause urinary tract infections. For this exam, a midstream specimen of urine is obtained in a sterile container after the genital area is washed. In people with IC, the urine is sterile and no bacterial growth is obtained.
- Cystoscopy with distention of the bladder: If no infectious agent is identified in the urine, cystoscopy is performed. In this procedure, the urologist uses a cystoscope (a hollow tube with a light source) to see the inside of the bladder. The bladder wall is stretched by filling it with liquid. This procedure may be performed under anesthesia because it may be painful. People with IC may have pinpoint hemorrhages, called glomerulations, in the bladder wall and/or ulcers (an open sore in the lining of the bladder), which can be viewed during the procedure.
- Biopsy of the bladder wall: A sample of the bladder wall tissue is removed for microscopic examination. This test also helps to rule out bladder cancer.
- Potassium sensitivity test: In this test, the urinary bladder is filled with either potassium solution or water, and pain and/or urgency scores are compared. A person who has IC feels more pain and/or urgency when the bladder is filled with potassium solution than when the bladder is filled with water. However, people with normal bladders cannot tell the difference between the two solutions.
What Is the Treatment for Interstitial Cystitis?
A range of treatment options for interstitial cystitis exists, from changing diet and exercise habits, to medication, to surgery. Each medical treatment comes with its own benefits and drawbacks, and a doctor recommends it based on the how the disease manifests itself.
What Are Home Remedies for Interstitial Cystitis?
Diet
Certain food items may worsen the symptoms of IC; they include the following:
- Citrus fruits
- Tomatoes
- Chocolates
- Coffee (or any caffeine)
- Spicy foods
- Artificial sweeteners
- Carbonated beverages
- Alcoholic beverages
All food items do not affect all people with IC in the same way. Therefore, each person should find out which food item makes one's symptoms worse. This can be done by trying an "elimination diet." On an elimination diet, one needs to stop eating all food items that can make symptoms worse. If symptoms improve on the elimination diet, the food item that was irritating the bladder needs to be identified. This can be done by introducing one food item at a time into the diet. If the addition of the food item does not worsen symptoms, it can be added to the regular diet. In this manner, one can identify the food item that makes symptoms worse and thus avoid it.
Smoking
Many people with IC have reported that smoking makes their symptoms worse. Quitting smoking will not only provide symptomatic relief to people with IC but will also decrease the risk of developing bladder cancer, because smoking is a known cause of bladder cancer. Smoking cessation will also decrease the incidence of heart disease, hypertension, stroke, peripheral vascular disease, and lung cancer.
Exercise
Many people with IC have reported that gentle stretching exercises help alleviate the symptoms of IC.
Bladder Training
People with IC may be able to reduce urinary frequency by using bladder-training techniques. They are advised to progressively increase the voiding (emptying the bladder) interval over the course of weeks to months by using relaxation techniques and distractions. A diary can help track the progress.
What Is the Medical Treatment for Interstitial Cystitis?
There is no cure for IC. The goal of treatment of IC is to provide relief of symptoms. Because there are probably many different causes of IC, no single treatment is effective for all people with IC. Treatment is tailored to the individual, based on the symptoms. Usually, different treatments are tried until symptoms improve.
People with IC may have flare-ups and remissions. A particular treatment may work for a time and then quit working. Sometimes, a change of diet or stress triggers the symptoms.
Most people with IC are helped by one or more of the following treatments:
- Dietary and smoking restrictions: Elimination of food items that worsen symptoms
- Medications: Anticholinergics, antimuscarinics, sodium pentosan polysulfate, tricyclic antidepressants, antihistamines, nonsteroidal antiinflammatory drugs
- Bladder instillations of drugs: Dimethyl sulfoxide, heparin, corticosteroids
- Procedures, such as hydrodistention with cystoscopy (stretching of bladder)
- Surgery
- Other therapies: Transcutaneous electrical nerve stimulation, sacral nerve stimulation, biofeedback
It is important to remember that no treatment works immediately. It may take weeks to months for symptoms to improve. Most people need to continue treatment for their whole lives because it is possible for IC symptoms to recur, even if the disease has been in remission for a long time.
What Are the Medications for Interstitial Cystitis?
Oral Therapy
Drugs should be considered after conservative measures have failed to provide substantial improvement in symptoms.
- Sodium pentosan polysulfate (Elmiron) is the only oral drug approved by the U.S. Food and Drug Administration (FDA) for the treatment of people with IC. Its mode of action is not entirely understood, but it may act as an antiinflammatory agent. Because it is structurally similar to naturally occurring glucosaminoglycans, it is believed to restore the protective layer on the bladder epithelium. Sodium pentosan polysulfate also has some anticoagulant action, and caution should be used when other anticoagulants are given. The dosage is 100 mg orally three times a day. Clinical studies suggest that maximal effects are not observed until the drug has been taken for at least five to six months. Side effects of sodium pentosan polysulfate include headache, rash, dizziness, diarrhea, dyspepsia, abdominal pain, hair loss (which is reversible), and liver function abnormalities.
- Tricyclic antidepressants (amitriptyline [Elavil, 25-75 mg at bedtime], doxepin [Adapin, Sinequan, 75 mg at bedtime], and imipramine [Tofranil, 25 mg three times a day]) are used in people with IC for their pain-relieving effects. They alleviate both the pain and frequency of IC and also help deal with the psychological stress associated with a chronically painful condition. They also cause drowsiness and deepen REM sleep, which helps in decreasing nocturia.
- Antihistamines can be helpful in treating IC. Hydroxyzine (Atarax, Vistaril, 25-75 mg at bedtime) and cimetidine (Tagamet, 300 mg twice daily) are the only antihistaminics that have been specifically used for the treatment of people with IC. The main side effect of hydroxyzine is sedation, which is actually a benefit because it helps the person with IC to sleep better at night and get up to urinate less frequently.
- Anticholinergics and antimuscarinics are the mainstay therapy for overactive bladder, urgency, and urge incontinence. They have a central role in IC. Tolteradine (Detrol), oxybutynin (Ditropan), and others are used extensively with good results and few side effects. High doses may be required, and combination therapy may be effective.
Bladder Drug Instillation (Bladder Wash)
- Dimethyl sulfoxide (DMSO, Rimso-50) is the only drug approved by the FDA for use in bladder instillation. Using a catheter, the bladder is filled with DMSO, which is retained in the bladder for 15-20 minutes before being emptied. The technique does not require anesthesia, hospitalization, or the use of an operating room. This treatment is given every week or two weeks for six to eight weeks. DMSO is believed to work as an antiinflammatory agent and therefore reduces pain. It may also prevent contractions that cause pain, frequency, and urgency. By the end of the sessions, complete relief of symptoms is often obtained.
If symptoms recur, more treatments can be given. People who are willing to catheterize themselves may be able to self-administer treatments at home. Side effects include a garlic-like body odor in some people. For some people, DMSO instillations can be painful. This can often be relieved by first instilling a local anesthetic into the bladder through a catheter or by mixing the local anesthetic with DMSO. Some clinicians substitute intravesical (instilled in the bladder) heparin for DMSO. Other agents can be added to DMSO making an IC "cocktail." These include corticosteroids, heparin, normal saline (sodium chloride solution), and lidocaine.
Is Surgery a Treatment for Interstitial Cystitis?
For those whose symptoms are severe and who do not respond to other IC treatments, bladder surgery may be considered. However, surgery does not necessarily improve symptoms. Several approaches and techniques have been used.
- Fulguration: This procedure involves burning ulcers, if present, with a laser by inserting instruments into the bladder through the urethra.
- Resection: This procedure involves cutting and removing the ulcer, if present, by inserting instruments into the bladder through the urethra.
- Augmentation: In this procedure, the scarred and ulcerated part of the bladder is removed and a piece of intestine (either large or small) is attached to the bladder. However, IC can sometimes recur on the segment of bowel used to augment the bladder. After the procedure, the person may have additional problems such as infections in the newly created bladder, incontinence, or they may require a catheter to empty the bladder.
- Cystectomy (bladder removal): After removal of the bladder, different procedures can be used to reroute the urine. Still, IC symptoms remain in as many as half of the patients after major surgery such as cystectomy. Detailed and honest counseling is imperative in these patients.
- In most people undergoing cystectomy, ureters (tubes that carry urine from each kidney to the bladder) are attached to a piece of intestine that opens onto the skin of the abdomen. Urine empties through the stoma (opening) into a bag outside the body. This procedure is commonly referred to as an ileal conduit.
- Some surgeons use a technique that allows urine to be stored in a pouch inside the abdomen, which can be emptied at intervals using a catheter. However, both this procedure and the ileal conduit have a potential for complications like kidney infection or kidney stones.
- Alternatively, a new bladder can be created from a piece of bowel and attached to the urethra. After healing, the person may be able to empty the bladder by voiding at regular intervals or by inserting a catheter or voiding spontaneously. Surgeons performing this procedure require special training and expertise.
- Strangely, even after total bladder removal, some people may still experience symptoms; therefore, surgery should only be considered after all alternative treatments have failed.
Other Therapies for Interstitial Cystitis
- Transcutaneous electrical nerve stimulation (TENS) is delivered through a device that is worn externally. The device delivers mild electric pulses to the bladder area and helps relieve pain and urinary frequency in some people with IC.
- Bladder distention: The urinary bladder is stretched by filling it with water under general anesthesia. This test is used for diagnosing IC and may provide relief as well. Bladder distention provides relief for some people with IC, at least over the short term, probably because the bladder is stretched and its capacity is increased. The procedure may interfere with the transmission of pain signals by nerves in the bladder and thereby provide pain relief. Symptoms may temporarily worsen 24-48 hours after bladder distention but usually improve two to four weeks after the procedure.
- Sacral nerve stimulation implants are surgically implanted devices being tested for people with IC.
- Self-help strategies such as bladder training, dietary changes, stress reduction, and low-impact exercise may help alleviate the symptoms of IC.
- Physical therapy with biofeedback for pelvic floor relaxation may be helpful in some people.
What Is the Prognosis for Interstitial Cystitis?
Outlook
IC is a condition with a variable course. For many people, the severity of symptoms fluctuates. For some, the condition goes into periods of remission. Rarely, some people may experience rapidly worsening symptoms. Although there is no treatment that reliably eliminates the symptoms of IC, a number of medications and therapies offer relief.
Support Groups and Counseling
IC has a profound adverse effect on the quality of life. Joining a support group may be especially helpful. A local chapter of the Interstitial Cystitis Association can help in providing a continuing network of support. Support and attention to associated psychosocial problems can greatly improve the person's response to treatment.
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REFERENCE:
"Pathogenesis, clinical features, and diagnosis of interstitial cystitis/bladder pain syndrome." UpToDate.com.