Interstitial Cystitis

U.S. Prevelance: 450,000 to 700,000 (52-67 per 100,000)
Women account for 90% of patients
Jewish persons account for 15% of patients
Age Distribution
1. Less commonly occurs in children
2. Onset between ages 30 to 70 years old
3. Patients under age 30 account for 25% of cases
4. Median age: 40 to 43 years old

Urothelium damage is the primary underlying problem in Interstitial Cystitis
Glycosaminoglycan deficiency in Bladder mucin layer
1. Hydrophilic and anionic mucous layer is water barrier for urothelium
2. Glycosaminoglycan deficiency disrupts protection
3. Possible response to prior Bacterial Urinary Tract Infection
Allows urinary solutes (especially Potassium) to provoke inflammation
1. Tissue irritation and injury
2. Sensory Nerve depolarization
3. Mast Cell degranulation
  1. Mast Cells may also be abnormal

Most common Symptoms
1. Dysuria
2. Dyspareunia
3. Suprapubic Pain or Pelvic Pain
  1. Relieved with small volume voids
  2. Pain recurs with Bladder filling
Other common symptoms
1. Excessive urinary urgency
  1. Uncomfortable constant urge to void
  2. Not relieved with voiding
2. Urinary Frequency
  1. More than 8 voids per day
  2. Average: 16 voids per day
  3. Reported as high as 40 voids per day
3. Nocturia
Infrequently associated symptoms
1. Gross Hematuria (20%)
Timing
1. Symptoms persist over at least 9 months (no longer required to make diagnosis)
2. Symptoms worse during week before Menses
Consider other diagnosis
1. Symptoms not due to recent Urinary Tract Infection
2. Incontinence suggests other diagnosis

Pelvic tenderness
1. Suprapubic tenderness
2. Tenderness on bimanual pelvic exam
Vaginal tenderness
1. Especially incolving lateral and anterior wall
2. Painful speculum exam
Rectal Pain
1. Rectal spasms or pain occur on Digital Rectal Exam
Decreased Bladder capacity
1. Bladder capacity under 350 ml (normal adult maximal capacity is ~1150 ml)
2. Urge to void occurs if Bladder distended >150 ml

Intravesical Potassium Sensitivity Test
1. Insert #10 french pediatric Feeding Tube into Bladder
2. Slowly instill 40 ml sterile water over 2-3 minutes and rank urgency and pain on scale of 0 to 5
3. Drain Bladder
4. Instill 40 ml of 40 meq KCL in 100 ml sterile water and rank urgency and pain on scale of 0 to 5
  1. No pain: Reassess after 5 minutes, then drain Bladder
  2. Pain: Drain Bladder, irrigate with 60 ml sterile water, followed with bladder Anesthetic (see below)
Anesthetic Bladder Challenge
1. Consider immediately after the intravesical Potassium sensitivity test (see above)
2. Insert #10 french pediatric Feeding Tube into Bladder
3. Instill Lidocaine 2% (10 ml) with bicarbonate 8.4% (4 ml) and Heparin 40,000 Units
4. Assess pain relief

Careful examination including pelvic exam
Patient keeps 24 hour log of voiding
Urodynamic Studies
1. Shows decreased Bladder capacity (reduced to <350 in Interstitial Cystitis)
2. Not specific for Interstitial Cystitis
Cystoscopy
1. Direct visualization
  1. May be helpful in evaluating for alternative diagnosis or assessing severity
  2. Not required for Interstitial Cystitis diagnosis
2. Hydrodistention (not required for Interstitial Cystitis diagnosis)
  1. Requires Anesthesia
  2. Identifies reduced Bladder capacity (normal approaches 1150 in healthy adults)
    1. Not specific for Interstitial Cystitis
  3. Risk of Urethral tears and Bladder perforation (rare)
3. Hunner's Ulcers
  1. Mucosal Ulcers on Bladder wall with granulation
  2. Brownish red ulcers involve all Bladder wall layers
4. Glomerulations on hydrodistention with saline
  1. Multiple petechial-like Hemorrhages in mucosa
  2. May be seen in asymptomatic patients
  3. Blood tinged fluid occurs in 90% of patients
5. Biopsy
  1. Not routinely done in U.S. unless concerns regarding possible Bladder Cancer
  2. Evaluate for neoplasia, dysplasia or Tuberculosis
  3. Confirms Bladder wall inflammation and may identify subgroups (e.g. Eosinophil excess)

Reassurance
1. Not cancer
2. Not indicator for more severe systemic disease
3. Therapy is symptomatic not curative
Avoid exacerbating foods
1. Coffee
2. Alcohol
3. Carbonated beverages
4. Citrus fruits or beverages
5. Artificial Sweeteners
6. Tomatoes
7. Chocolate
Chronic Pain Management adjuncts
1. Support groups (See resources below)
2. Transcutaneous electrical nerve stimulation (TENS)
3. Sacral nerve stimulation or pudendal nerve stimulation
4. Physical Therapy with biofeedback
  1. Pelvic floor relaxation Exercises

Pentosan polysulfate (Elmiron)
1. Replaces epithelial function
2. Dose: 300-400 mg orally daily divided two to three times daily
3. Risk of Retinal damage (Pigmentary Maculopathy) with prolonged use (typically with years of use)
Tricyclic Antidepressants
1. Inhibits Neuron activation
2. Amitriptyline (Elavil) or Nortriptyline (Pamelor)
3. Dose start: 10-25 mg orally at bedtime
4. Titrate to 50-75 mg orally at bedtime
Hydroxazine (Atarax)
1. Dose: 25-50 mg orally at bedtime
2. May reduce Mast Cell degranulation symptoms

Instillation Agents
1. Dimethyl sulfoxide (Rimso-50) 50% solution every 1-2 weeks for 6-8 times
  1. Heparin 10,000 Unit 3x/week (may potentiate Rimso-50)
2. Hyaluronic acid 40 mg weekly
3. Bacillus Calmette-Guerin (BCG) weekly for 6 weeks
Administration
1. Initially performed in clinic via Urinary Catheter
2. Patient may learn to self-catheterize for home
Efficacy
1. Long-term remission seen in >50% of patients