Interstitial Cystitis/Bladder Pain Syndrome

Interstitial Cystitis or Painful Bladder Syndrome is an unpleasant sensation (pain, pressure, discomfort) perceived to be related to the urinary bladder, associated with lower urinary tract symptoms of more than 6 weeks’ duration, in the absence of infection or other identifiable causes.

People with IC have inflamed or irritated bladder walls, which can cause scarring and stiffening of the bladder. According to the cystoscopy appearance, we distinguish two main IC groups:

Non-ulcerative (90%): the cystoscopy shows pinpoint haemorrhages in the bladder wall, also known as glomerulations. However, these are not specific to IC, and any bladder inflammation may have these lesions.

Ulcerative (5-10%): these patients usually have Hunner’s ulcers or patches, which are red, bleeding areas on the bladder wall.

While it is prevalent in women (up to 12% of all women in the United States), IC is rare in children. In men, symptoms of IC may be confused with other common conditions like chronic prostatitis.

Causes of Interstitial Cystitis

The exact cause of IC is unknown. However, there are several hypotheses regarding why IC/PBS may occur. We suspect a possible multifactorial process leading to the bladder damage. The result is microscopic wall gaps, where urine particles such as potassium and acidic ions may leak into the bladder wall making inflammation/irritation of the underlying sensitive nerve branches and causing chronic nerve pain/irritative bladder symptoms (frequent and urgent urination). According to this theory, the damage may occur with (risk factors for IC):

• Bladder trauma (such as from pelvic surgery)
• Bladder overdistention (anecdotal cases suggest onset after prolonged periods without access to bathroom facilities)
• Pelvic floor muscle dysfunction
• Autoimmune disorder
• Bacterial infection (Cystitis)
• Primary neurogenic inflammation (hypersensitivity or inflammation of pelvic nerves)
• Spinal cord trauma

Interstitial Cystitis and Bladder Pain Syndrome may also be associated with chronic conditions such as fibromyalgia, myalgic encephalomyelitis or chronic fatigue syndrome (ME/CFS) and irritable bowel syndrome (IBS).

Symptoms

• The pelvic pain or bladder pain
• Frequent urination
• Urgent urination

All these symptoms may have different severity among patients. According to the prevalence of one sign to others, we can identify different subtypes (phenotypes) of IC/BPS.

The pain in Interstitial Cystitis

The description of pain in IC/BPS deserves a thorough discussion. With pelvic pain, we generally define unpleasant sensory and emotional experiences associated with bladder inflammation. It occurs mainly in the lower abdomen or the perineum (the area between anus and vagina or scrotum). It intensifies as the urinary bladder fills or empties. This pain might be steady or intermittent. It can be a sharp stabbing in a specific spot or a dull ache spread out. The pain is usually chronic, so that recurs frequently or lasts for a longer time. In females often, the pain occurs during sexual intercourse (dyspareunia).

Tool to help the patient to describe his pain

• Where you feel pain;
• What it feels like – some words to use are*:
  • Sharp – like a cut;
  • Dull – like a sore muscle or muscle ache;
  • Throbbing;
  • Steady;
  • Burning;
  • Shooting;
• How severe the pain is – use the 0-to-10 pain scale described in the picture;
• How long the pain lasts – for example, minutes, hours, days;
• What relieves the pain – for instance, cold compresses, heat, repositioning, medicines^;
• What makes the pain worse – for example, moving, changing position, it gets worse in the evening, etc.;
• What pain medicines are you taking – when do you take them, and how much relief do you get from them.

Tool to help the patient to rate his pain

Using a visual analogue scale helps to describe the intensity of pain. For example, the patient can rate it with a number from “0” to “10”. “Zero” means no pain, while “10” means the worst pain ever felt.

Table 1: Visual Analog Scale to rate the pelvic pain

The patient can use this 0-10 rating scale to describe:

• How bad the pain is at its worst;
• How bad the pain is most of the time;
• How bad the pain is, at its least.

However, it can be hard to remember the details every time pain occurs. It is helpful to use a pain diary to describe and record the pain episodes when they occur.

Table 2: Pain diary

Frequent urination

Patients may void insignificant amounts every 20-30 minutes and even at night. We can only imagine how symptoms might impact daily activities and night sleep.

Urgent Urination

The feeling of a sudden urge to urinate is challenging to control.

Diagnosis

There is no standardized test for interstitial cystitis/painful bladder syndrome diagnosis. The symptoms make the diagnosis in the Interstitial Cystitis or Blabber Pain Syndrome. When the patient complains about pelvic pain, or irritative bladder symptoms (frequent and urgent urination), we must rule out other treatable conditions with similar symptoms before considering a diagnosis of IC/BPS. Once we exclude different diseases, we treat patients for presumed IC.

Diseases with similar symptoms to be differentiated

There is a list of confoundable chronic diseases we must rule out before starting to treat IC/BPS:

• Bladder cancer
• Highly recurrent UTI
• Other infections
  • Candida
  • Mycobacterium tuberculosis
• STDs
• overactive bladder
• chronic prostatitis
• prostate enlargement
• endometriosis
• bladder and lower ureteral stones
• Pelvic radiation and chemotherapy
• Genital cancers
• Urogenital prolapse
• Pudendal nerve entrapment
• Pelvic-floor muscle-related pain
• Bladder-neck obstruction
• Pelvic Inflammatory Disease (PID)

Then, the whole diagnostic is a multidisciplinary workup that involves the urologist and gynaecologist. Moreover, since food intolerance and IBS may be concomitant, the gastroenterologist may be included in the patient’s evaluation.

Diagnostic workup

Urologist:

• rectal examination to suspect pudendal nerve entrapment and pelvic-floor muscle-related pain;
• Urine analysis;
• Urine culture if pyuria and leucocyte esterase;
• STD panel and Ziehl-Neelsen stain (for tuberculosis), if leucocyte esterase and sterile pyuria;
• Urine cytology: to identify possible bladder cancer;
• Imaging urinary tract and pelvis US scan: to detect bladder and genital cancer, stones, bladder abnormalities (thick bladder walls); suspect endometriosis.
• Urodynamics and uroflowmetry: to assess bladder obstruction, to confirm overactive bladder;

Gynaecology referral to exclude:

• Genital cancers;
• Endometriosis;
• Genital prolapse;
• Pelvic Inflammatory Disease (PID).

Gastroenterologist

• food intolerance and allergy
• IBS

Specific diagnostics for IC/BPS

When we rule out all confoundable diseases, we may consider some diagnostics to confirm a presumed IC/BPS.

• Cystoscopy with or without biopsy;
• Lidocaine bladder instillation test;
• food intolerance and allergy test.

Questionnaires to diagnose IC/BPS

Two symptom screening questionnaires are available: the O’Leary-Sant Symptom and Problem Index and the Pelvic Pain and Urgency/Frequency Symptom Scale (PUF).

Physical examination

The examination includes a bimanual (vaginal-lower abdominal) pelvic examination in women and rectal examination in men. Assessment of patients with interstitial cystitis/painful bladder syndrome may have pelvic floor spasms, rectal spasms, or suprapubic tenderness. Women may feel tenderness in the anterior vaginal wall and bladder base. After physical examination, we can suspect two possible confoundable diseases (pudendal-nerve entrapment and pelvic-muscle-related pain).

Intravesical Potassium Sensitivity Test

The Potassium Chloride Sensitivity Test (PST), or Parson’s test, consists of a bladder instillation of potassium chloride through a urinary catheter. The PST is no longer widely used because of its low sensitivity and specificity and because it could be painful and trigger a pelvic pain crisis.

Local anaesthetic bladder instillation

We can install a local anaesthetic solution in a symptomatic patient through a catheter. If the bladder is the source of pain, the patient will experience significant pain relief.

Cystoscopy and hydrodistension

Cystoscopy is a mild invasive diagnostic that allows us to visualize the internal bladder walls directly and document the bladder inflammation and its severity (the presence of ulceration). During cystoscopy, we can perform the hydrodistension, which consists of filling the bladder with water until the maximum bladder capacity to detect mucosal changes considered typical of IC/BPS. Moreover, hydrodistension can aid in evaluating maximal bladder capacity (about 1,150 mL in healthy adults). The maximum bladder capacity is low or very low in severe symptomatic cases. This finding may help us to confirm IC/IBS.

However, due to the low level of evidence, we do not recommend cystoscopy in all cases (in mildly symptomatic patients, for example).

Bladder biopsy

We do not perform bladder biopsies routinely. We do it if we suspect symptomatic bladder cancer (carcinoma in-situ).

Urodynamics

Urodynamics are not required but may help differentiate interstitial cystitis/painful bladder syndrome from detrusor overactivity.

Treatment options

We do not know the cause of IC/BPS. That is why the therapy includes an empiric combination of treatments to improve the symptoms and the quality of life. This treatment strategy includes lifestyle modifications (stress management), dietary restrictions, food supplements, medications, intravesical treatments and complementary therapies. It is essential to know that we tailor the treatment strategy to the patient, and the response to a cure is variable and not reproducible among different patients.

Eating, Diet, & Nutrition for Interstitial Cystitis

Some research strongly suggests a relationship between symptoms and diet. However, more than recommending a diet to help relieve symptoms of IC/BPS, we should focus on the restriction of known trigger foods. Some patients are aware of symptoms triggering or worsening after eating certain foods. Coffee, soda, alcohol, tomatoes, hot and spicy foods, chocolate, caffeinated beverages, citrus juices and drinks, monosodium glutamate, and high-acid foods can trigger or worsen IC symptoms. Some people also note that their symptoms get worse after eating or drinking products with artificial sweeteners like soft drinks. The diet diary may help patients link the symptoms flares to specific foods.

The elimination diet is an eating plan that stops certain foods and drinks one at a time. By removing certain foods for a period and then reintroducing them during a “challenge” period, we can learn which foods are causing symptoms.

Antiacids taken with meals reduce the urine acidity related to some foods and may relieve the symptoms.

We must assess the patient for food intolerance and allergy which might trigger IC/BPS symptoms.

Oral medications

Pentosan polysulfate sodium (Elmiron) is the only oral therapy approved by the US Food and Drug Administration (FDA) for treating interstitial Cystitis.

Other medications are only empiric and include:

• Amitriptyline;
• hydroxyzine (Vistaril);
• cimetidine (Tagamet);
• cyclosporine A (Sandimmune);
• doxycycline;
• urinary anaesthetic (phenazopyridine [Pyridium]);
• alpha-blockers;
• benzodiazepines;
• muscle relaxants;

Intravesical therapies

Dimethyl sulfoxide is the only FDA-approved intravesical agent to treat painful symptoms of interstitial cystitis/painful bladder syndrome. The bladder irrigation with 50% dimethyl sulfoxide solution is used for six to eight weeks to relieve moderate to severe pain symptoms of IC/BPS.

We can use Pentosan polysulfate sodium (Elmiron) in bladder instillations alone or combined with the oral formulation.

Heparin and hyaluronic acid (combined with chondroitin sulfate) are other possible medications used for intravesical therapy.

Other therapies

Sacral nerve stimulation may be effective only for frequency associated with IC/BPS but not for pain relief.

We can obtain better results in terms of pain control with pudendal nerve stimulation.

We use physical therapy in select cases of IC/BPS, especially for treating associated pelvic floor muscle spasms.

External resources

Healing Interstitial Cystitis

Interstitial Cystitis Association