Interstitial Cystitis

para>Unpredictable symptom improvement or exacerbation during pregnancy; no known fetal effects from interstitial cystitis; usual problems of unknown effect on fetus with medications taken during pregnancy

EPIDEMIOLOGY

Occurs predominantly among whites
Predominant sex: female > male (10:1)
Patients <30 years have predominant symptoms: dysuria, frequency, urinary urgency, pain in external genitals, and dyspareunia; and those >60 years more commonly have nocturia, urinary incontinence, or Hunner ulcer disease.
Predominant age
- Mild: 20 to 40 years
- Severe: 20 to 70 years
Pediatric considerations
- <10 years old and again at 13 to 17 years
- Daytime enuresis, dysuria without infection

Prevalence
In the United States:

Up to 1 million affected, but many cases likely are unreported
0.052% but may be higher up to 10%

ETIOLOGY AND PATHOPHYSIOLOGY

Unknown but is not primarily psychosomatic
Possible causes
- Subclinical urinary infection
- Damage to glycosaminoglycan mucus layer increasing bladder wall permeability to irritants such as urea
- Autoimmune
- Mast cell histamine release
Neurologic upregulation/stimulation

RISK FACTORS

Unknown

COMMONLY ASSOCIATED CONDITIONS

Fibromyalgia
Allergies
Chronic fatigue syndrome
Depression
Vulvodynia
Sexual dysfunction
Sleep disturbance
Migraines
Syncope
Dyspepsia
Chronic prostatitis
Chronic pelvic pain
Irritable bowel syndrome
Anal/rectal disease

DIAGNOSIS

Frequent, urgent, relentless urination day and night; >8 voids in 24 hours
Pain with full bladder that resolves with bladder emptying (except if bacteriuria is present)
Urge urinary incontinence if bladder capacity is small
Sleep disturbance
Dyspareunia, especially with full bladder
Secondary symptoms from chronic pain and sleeplessness, especially depression

HISTORY

Pelvic Pain and Urgency/Frequency Patient Symptom Scale: self-reporting questionnaire for screening potential interstitial cystitis patients (1)[B] (http://www.wgcaobgyn.com/files/urgency_frequency_pt_symptom_scale.pdf)
Frequent UTIs, vaginitis, or symptoms during the week before menses
O'Leary/Sant Voiding and Pain Indices (http://www.ichelp.org/wp-content/uploads/2015/06/OLeary_Sant.pdf)

PHYSICAL EXAM

Perineal/prostatic pain in men
Anterior vaginal wall pain in women

DIFFERENTIAL DIAGNOSIS

Uninhibited bladder (urgency, frequency, urge incontinence, less pain, symptoms usually decrease when asleep)
Urinary infection: cystitis, prostatitis
Bladder neoplasm
Bladder stone
Neurologic bladder disease
Nonurinary pelvic disease (STIs, endometriosis, pelvic relaxation)

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests (lab, imaging)

Urinalysis: normal except with chronic bacteriuria (rare)
Urine culture from catheterized specimen: normal except with chronic bacteriuria (rare) or partial antibiotic treatment
Urine cytology
- Normal: reserve for men >40 years old and women with hematuria

Diagnostic Procedures/Other

Cystoscopy (especially in men >40 years old or women with hematuria)
- Bladder wall visualization
- Hydraulic distention: no improved diagnostic certainty over history and physical alone
No role for urodynamic testing
Intravesical lidocaine can help to pinpoint the bladder as the source of pain in patients with pelvic pain; this can be both diagnostic and therapeutic.
Potassium sensitivity test
- Insert catheter, empty bladder, instill 40 mL H2O over 2 to 3 minutes, rank urgency on scale of 0 to 5 in intensity, rank pain on scale of 0 to 5 in intensity, drain bladder, instill 40 mL potassium chloride (KCl) 0.4 mol/L solution:
  - If immediate pain, flush bladder with 60 mL H2O and treat with bladder instillations.
  - If no immediate pain, wait for 5 minutes and rate the urgency and pain.
If urgency or pain >2, treat as above.
Pain or urgency >2 is considered a positive test and strongly correlates with interstitial cystitis if no radiation cystitis or acute bacterial cystitis is present.

Test Interpretation

Nonspecific chronic inflammation on bladder biopsies
Urine cytology negative for dysplasia and neoplasia
Possible mast cell proliferation in mucosa

TREATMENT

GENERAL MEASURES

Appropriate health care: outpatient
Self-care (eliminate foods and liquids that exacerbate symptoms on individual basis, fluid management) (2)[C]
Biofeedback bladder retraining (2)[C]

MEDICATION

Randomized controlled trials of most medications for interstitial cystitis demonstrate limited benefit over placebo; there are no clear predictors of what will benefit an individual. Prepare the patient that treatment may involve trial and error.
Behavioral therapy combined with oral agents found improved outcomes compared to medications alone.
Intravesical injections of botulinum toxin are not effective in the treatment of ulcer-type interstitial cystitis.

First Line

Note: AUA consensus states medicines should be considered second-line therapy after patient education, stress reduction, behavior modification, and self-care (2)[C].
Pentosan polysulfate (Elmiron) 100 mg TID on empty stomach; may take several months (3 to 6) to become effective; rated as modestly beneficial in systematic drug review (only FDA-approved treatment for interstitial cystitis) (3)[C]
Amitriptyline: Most effective at higher doses (≥50 mg/day); however, initiate with lower doses to minimize side effects (4)[B].
Hydroxyzine 25 to 50 mg HS
Sildenafil 25 mg/day (5)[B]
Cimetidine 400 mg BID (2)[C]
Triple-drug therapy: 6 months of pentosan, hydroxyzine, doxepin
Antibacterials for bacteriuria
Oxybutynin, hyoscyamine, tolterodine, and other anticholinergic medications decrease frequency.
Prednisone (only for ulcerative lesions)
Montelukast has shown some benefit.
NSAIDs for pain and any inflammatory component
Bladder instillations
- Lidocaine, sodium bicarbonate, and heparin or pentosan polysulfate sodium
- Dimethyl sulfoxide (DMSO) every 1 to 2 weeks for 3 to 6 weeks, then PRN
- Heparin sometimes added to DMSO
- Intravesical liposomes
- Other agents: steroids, silver nitrate, oxychlorosene (Clorpactin)
Contraindication
- No anticholinergics for patients with close-angle glaucoma
Significant possible interaction
- Refer to manufacturer's profile of each drug.

Second Line

Phenazopyridine, a local bladder mucosal anesthetic, usually is not very effective.
Intravesicular injection of botulinum type A for nonulcer interstitial cystitis
Cyclosporin A (2)[C]

ISSUES FOR REFERRAL

Need for clarity with respect to diagnosis
Surgical intervention

ADDITIONAL THERAPIES

Myofascial physical therapy (targeted pelvic, hip girdle, abdominal trigger point massage) (6)[B]

SURGERY/OTHER PROCEDURES

Hydraulic distention of bladder under anesthesia: symptomatic but transient relief
Cauterization of bladder ulcer
Augmentation cystoplasty to increase bladder capacity and decrease pressure with or without partial cystectomy. Expected results in severe cases: much improved, 75%; with residual discomfort, 20%; unchanged, 5%
Urinary diversion with total cystectomy only if disease is completely refractory to medical therapy
Sacral neuromodulation

COMPLEMENTARY & ALTERNATIVE MEDICINE

Guided imagery

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring
Not specifically needed unless symptoms are unresponsive to treatment

DIET

Variable effects from person to person
Common irritants include caffeine, chocolate, citrus, tomatoes, carbonated beverages, potassium-rich foods, spicy foods, acidic foods, and alcohol.

PATIENT EDUCATION

Interstitial Cystitis Association, 110 Washington St. Suite 340, Rockville, MD 20850; 1-800-HELPICA: http://www.ichelp.org/

PROGNOSIS

Mild: exacerbations and remissions of symptoms; may not be progressive; does not predispose to other diseases
Severe: progressive problems that usually require surgery to control symptoms

COMPLICATIONS

Severe, with long-term, continuous high bladder pressure could be associated with renal damage.

REFERENCES

11 Parsons CL, Dell J, Stanford EJ, et al. Increased prevalence of interstitial cystitis: previously unrecognized urologic and gynecological cases identified using a new symptom questionnaire and intravesical potassium sensitivity. Urology. 2002;60(4):573-578.22 Hanno PM, Erickson D, Moldwin R, et al. Diagnosis and treatment of interstitial cystitis/bladder pain syndrome: AUA guideline amendment. J Urol. 2015;193(5):1545-1553.33 Dancel R, Mounsey A, Handler L. Medications for treatment of interstitial cystitis. Am Fam Physician. 2015;91(2):116-118.44 Foster HEJr, Hanno PM, Nickel JC, et al. Effect of amitriptyline on symptoms in treatment na ¯ve patients with interstitial cystitis/painful bladder syndrome. J Urol. 2010;183(5):1853-1858.55 Chen H, Wang F, Chen W, et al. Efficacy of daily low-dose sildenafil for treating interstitial cystitis: results of a randomized, double-blind, placebo-controlled trial-treatment of interstitial cystitis/painful bladder syndrome with low-dose sildenafil. Urology. 2014;84(1):51-56.66 FitzGerald MP, Payne CK, Lukacz ES, et al. Randomized multicenter clinical trial of myofascial physical therapy in women with interstitial cystitis/painful bladder syndrome and pelvic floor tenderness. J Urol. 2012;187(6):2113-2118.

ADDITIONAL READING

Rais-Bahrami S, Friedlander JI, Herati AS, et al. Symptom profile variability of interstitial cystitis/painful bladder syndrome by age. BJU Int. 2012;109(9):1356-1359.

CODES

ICD10

N30.10 Interstitial cystitis (chronic) without hematuria
N30.11 Interstitial cystitis (chronic) with hematuria

ICD9

595.1 Chronic interstitial cystitis

SNOMED

197834003 Chronic interstitial cystitis (disorder)
79160001 Hunner's ulcer (disorder)
36139001 Chronic ulcerating interstitial cystitis (disorder)

CLINICAL PEARLS

The potassium sensitivity test has been the most useful in confirming an initial diagnosis of interstitial cystitis.
Potassium sensitivity test
- Insert catheter, empty bladder, instill 40 mL H2O over 2 to 3 minutes; rank urgency on scale of 0 to 5 in intensity; rank pain on scale of 0 to 5 in intensity; drain bladder; and instill 40 mL KCl 0.4 mol/L solution.
Submucosal petechial hemorrhages and/or ulceration at the time of bladder distention and cystoscopy further support the diagnosis.
At present, there is no definitive treatment for interstitial cystitis.
Most patients with severe disease receive multiple treatment approaches. Regular multidisciplinary follow-up, pharmacologic therapy, avoidance of symptom triggers, and psychological and supportive therapy are all important because this disease tends to wax and wane. Monitor patients for comorbid depression.
Empowering patients to manage their symptoms, communicate regularly with their physicians, and learn as much as they can about this disease which may help them to optimize their outcome.

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