Nikhil Waingankar, MD
Sonia Bahlani, MD
Robert M. Moldwin, MD, FACS
BASICS
DESCRIPTION
• Interstitial cystitis (IC) or Painful Bladder Syndrome (PBS) is an unpleasant sensation (pain, pressure, discomfort) perceived to be related to the bladder, associated with lower urinary tract symptoms (LUTS) for more than 6 wk duration, and in the absence of other identifiable causes (1,2)
• 92% of patients also complain of frequency (>10–12× daily); nocturia is common
• 84% complain of constant/persistent urgency
• Dysuria is uncommon
• Symptoms can be associated with a wide range of diseases (see “Differential Diagnosis”)
• Two forms of IC/BPS:
– “Classic”: Associated with Hunner lesions on cystoscopy (formerly known as Hunner ulcer)
– “Nonclassic”: No inflammatory lesions identified upon cystoscopy
• Nomenclature change from IC to IC/PBS due to the lack of gross inflammatory bladder wall changes found in most patients
• Median age of onset 30–40 yr
• Female: Male ∼ 5:1
• 5–10% of patients have Hunner lesions
EPIDEMIOLOGY (3)
Incidence and prevalence vary widely
Incidence
0.6–1.6 per 100,000 people
Prevalence
Ranges from 1.6 to 2,600 per 100,000 people
RISK FACTORS
No known risk factors beyond a possible genetic predisposition
Genetics
Adult female 1st-degree relatives of IC patients have a prevalence 17× greater than that of the general population
PATHOPHYSIOLOGY
• Multifactorial etiology with a number of proposed mechanisms
– Epithelial permeability
– Antiproliferative factor
– Mast cell activation
– Neurogenic inflammation
– Infectious
– Autoimmunity
– Urinary abnormality: Toxic, allergic, immunologic
ASSOCIATED CONDITIONS
• Myalgia of pelvic floor: Most commonly identified comorbid condition
• Irritable bowel syndrome
• Fibromyalgia
• Chronic fatigue syndrome
• Multiple allergies
• Sjögren syndrome
• Chronic headaches
• Depression/anxiety/panic disorder
• In females: Vulvodynia, endometriosis
• In males: Chronic prostatitis/chronic pelvic pain syndrome, BPH, prostate cancer
GENERAL PREVENTION
No definitive prevention strategies, although dietary changes and medical therapy may mitigate symptom flares
DIAGNOSIS
HISTORY
• IC patients 10× more likely to have childhood bladder problems
• Symptoms unrelated to any identifiable cause (infection, STD, cancer, radiation, overactive bladder (OAB), diverticula, vaginitis, stones)
• Chronic pelvic pain, pressure
• Abdominal/supra-pubic pain
• Pain associated with bladder filling and/or emptying
• Premenstrual flares
• Urinary frequency, urgency, nocturia
• Urinary frequency based upon need to decrease level of pelvic discomfort/pain
• Helpful evaluation/monitoring tools:
– Symptom evaluation with voiding diary
– O’Leary-Sant Symptom and Problem Score
– Visual analog scale (pain score)
– PUF (Pelvic Pain & Urgency/Frequency) Questionnaire
– Bladder Pain/Interstitial Cystitis Symptom Score
PHYSICAL EXAM
• General:
– Abdominal exam to assess for supra-pubic tenderness
– Focused neurologic exam
• Females:
– Q-tip test to assess for vulvodynia
– Bimanual exam with palpation of bladder, urethra, and pelvic floor muscles to assess presence of muscle tenderness/banding
• Males:
– Digital rectal exam with palpation of prostate and pelvic floor musculature
– External genitalia exam
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Urinalysis and urine culture
• Urine cytology in high-risk groups
Imaging
No recommended imaging for the diagnosis of IC/PBS
Diagnostic Procedures/Surgery
• Urodynamics:
– Normal detrusor function on cystometry; may have increased sensitivity (pain) on filling and decreased capacity.
– Late stages of “classic” IC/PBS may be associated with significant decrease in capacity and bladder compliance
• Cystoscopy:
– Used selectively to exclude other bladder pathology and identify Hunner lesions
• Cystoscopy with hydrodistention under general/spinal anesthesia:
– Findings may include: Glomerulations (small foci of hemorrhage), Hunner lesions, decreased anesthetic capacity, mucosal tears; low sensitivity and specificity
– Hunner lesion (ulcer) is described as circumscript, reddened area with small vessels radiating toward a central scar. Fibrin deposit/coagulum can be attached to this area. With bladder distention the site ruptures with petechial blood oozing from the lesion and mucosal margins (5)
• Potassium sensitivity testing (KCl test):
– Low sensitivity and specificity; positive result provokes pain
• Residual urine/flow in males
Pathologic Findings
• Histologic findings can vary widely and none are truly pathognomonic
• Bladder biopsy
– Indicated only to rule out other disease processes
– Hunner lesions demonstrate pan-mural inflammation
DIFFERENTIAL DIAGNOSIS
• Bacterial cystitis
• Bladder cancer (including CIS)
• Bladder effects of chemotherapy
• Bladder outlet obstruction/urinary retention
• Bladder/lower ureteral stone
• Genital herpes
• Overactive bladder
• Pelvic floor muscle dysfunction
• Pudendal nerve entrapment
• Radiation cystitis
• Females:
– Cervical/uterine/ovarian cancer
– Urethral diverticulum
– Pelvic organ prolapse
– Endometriosis
– Vaginal candidiasis
• Males: BPH, prostate cancer, prostatitis
TREATMENT
GENERAL MEASURES
(Adapted from AUA guidelines 2011) (6)
• Patients should be aware that no single treatment has been found effective
• 1st line
– Stress reduction
– Exercise
– Warm baths
– Stool softeners
– Biofeedback
– Avoidance of spicy foods, caffeine, alcohol, artificial sweetener, acidic beverages
• 2nd line
– Pelvic floor physical therapy/massage
– Multimodal pain management
– Amitriptyline
– Cimetidine
– Hydroxyzine
– Pentosan polysulfate
– Intravesical instillation: Author’s preferred “cocktail”: Lidocaine, gentamicin, heparin, triamcinolone
– Intravesical: 50% DMSO
– Intravesical: 4% alkalinized lidocaine
• 3rd line
– Cystoscopy with hydrodistention (low pressure/short duration)
– Fulguration of Hunner lesions
– Submucosal injection of Hunner lesions with triamcinolone
• 4th line
– Neuromodulation (InterStim, etc.)
• 5th line
– Cyclosporine A
– Intradetrusor botulinum toxin A
• 6th line
– Urinary diversion +/– cystectomy: May eliminate urinary frequency but does not necessarily eliminate the pain component
MEDICATION
First Line
See above
Second Line
See Above
SURGERY/OTHER PROCEDURES
See above
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
See General Measures
Complementary & Alternative Therapies
See General Measures; myofascial physical therapy may help (4)
ONGOING CARE
PROGNOSIS
Spontaneous remission rate of 50% at mean of 8 mo
COMPLICATIONS
N/A
FOLLOW-UP
Patient Monitoring
Follow symptoms
Patient Resources
Interstitial Cystitis Association http://www.ichelp.org/
REFERENCES
1. Hanno P, Lin AT, Nordling J, et al. Bladder pain syndrome. In: Abrams P, Cardozo L, Khoury S, Wein A, eds. Incontinence. Paris, France: Health Publication Ltd; 2009. pp. 1459–518.
2. Van de Merwe JP, Nordling J, Bouchelouche P, et al. Diagnostic criteria, classification, and nomenclature for painful bladder syndrome/ interstitial cystitis: An ESSIC proposal. Eur Urol. 2008;53:60–67.
3. Suskind AM, Berry SH, Ewing BA, et al. The prevalence and overlap of interstitial cystitis/bladder pain syndrome and chronic prostatitis/chronic pelvic pain syndrome in men: Results of the RAND Interstitial Cystitis Epidemiology male study. J Urol. 2013;189(1):141–145.
4. 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.
5. Hillelsohn J, Rais-Bahrami S, Friedlander JI, et al. Fulguration for Hunner ulcers: Long term clinical outcomes. J Urol. 2012;188(6):2238–2241.
6. Hanno P, Burks DA, Clemens JQ, et al. AUA guideline for the diagnosis and treatment of interstitial cystitis/bladder pain syndrome. J Urol. 2011;185(6):2162–2170.
ADDITIONAL READING
• Friedlander JI, Shorter B, Moldwin RM. Diet and its role in interstitial cystitis/bladder pain syndrome and comorbid conditions. BJU Int. 2012;109(11):1584–1591.
• The Interstitial Cystitis Survival Guide: Your guide to the latest treatment options and coping strategies. Moldwin RM. New Harbinger Publications, Oakland CA, Oct 2000.
• Understanding the IC/PBS Diet. Beyer J, Gordon B, Laumann B, Osborne J, Shorter B. ichelp.org.
See Also (Topic, Algorithm, Media)
• Interstitial Cystitis (IC)/Painful Bladder Syndrome (PBS) Image 
• Lower Urinary Tract Symptoms (LUTS)
• Pelvic Pain, Female
• Pelvic Pain, Male
• Prostatitis, General
CODES
ICD9
• 595.1 Chronic interstitial cystitis
• 599.70 Hematuria, unspecified
• 788.41 Urinary frequency
ICD10
• N30.10 Interstitial cystitis (chronic) without hematuria
• N30.11 Interstitial cystitis (chronic) with hematuria
• R35.0 Frequency of micturition
CLINICAL/SURGICAL PEARLS
• IC/PBS is more common in women than in men.
• This is primarily a clinical diagnosis based upon the presence of characteristic symptoms and the exclusion of other causes.