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Objectives:

* Discuss the new terminology and disease classification of interstitial cystitis (IC)
* Present data on epidemiology of the disease including its overlap with other pelvic organ syndromes
* Review current approaches to diagnosis
* Provide overview of current treatment approaches

Material and Methods:

Review of evidence-based literature and expert consensus-based opinion on interstitial cystitis. This is augmented by discussions with national and international experts on IC during recent IC international meetings and AUA annual meetings.

Introduction:

Interstitial cystitis (IC) is a heterogeneous bladder and pelvic organs syndrome of unknown etiology and is characterized by irritative voiding symptoms and pelvic pain. Symptoms overlap and are frequently confused with chronic pelvic pain, syndromes, prostatitis, overactive bladder (OAB), and chronic cystitis. Over recent years there have been attempts to reclassify and expand the nomenclature of IC- painful bladder syndrome (PBS), bladder pain syndrome (BPS).

The etiology and pathophysiology of IC/PBS/BPS remain unknown. However, it is generally accepted that the following are involved - sensory bladder afferent nervous systems with spinal cord and central nervous system upregulation, defective epithelial permeability secondary to a bladder surface glycosaminoglycans (GAGs) abnormality, a neuro-inflammatory component involving bladder mast cell activation. A possible immune biology is suggested by the overlap with diseases such as irritable bowel syndrome and Sjogren's syndrome.

Diagnosis:

IC/PBS/BPS should be suspected in patients with irritable voiding symptoms (frequency, nocturia) and bladder/pelvic pain in whom specific bladder and pelvic diseases have been excluded. Symptoms can overlap and occur in conjunction with gynecologic causes of chronic pelvic pain (CPP), chronic prostatitis/chronic pelvic pain syndromes (CP/CPPS) in men, OAB and chronic bacterial cystitis.

Clinical diagnosis is by exclusion of specific disease in the face of symptoms as assessed by history or one of the IC- related self-administered patient questionnaires e.g.; O'Leary-Sant or Pain/Urgency/Frequency. Cystoscopy and hydrodistension excludes specific diseases such as carcinoma-in-situ, radiation cystitis, assesses bladder capacity and degree of mast cell involvement and activation. Glomerulations are a tell-tale feature of IC and about 5% patient will have severely reduced bladder capacity and Hunner's ulcers.

Treatment:

General supportive measures include dietary modification with limitation of alcohol, citrus/tomatoes, coffee, spices, citric acid and carbonated beverages. Psychological and behavioral modifications are also useful. Oral drugs are beneficial in treatment of IC/PBS/BPS- amitriptyline, the synthetic GAG sodium pentosanpolysulfate (Elmiron), the anti-histamine Hydroxyzine and anti-muscarinic agents. Frequently these drugs are used in a combination multi-modality treatment approach. Intravesical therapies include heparin, dimethyl sulfoxide, Bacillus Calmette-Guerin (BCG), lidocaine, bicarbonate and steroids. Complimentary treatments include pelvic floor manual massage therapy, relaxations techniques, acupuncture etc. Neuromodulation with nerve stimulators (S3, pudendal) can be helpful. Rarely is major surgical reconstruction indicated. Pain management is a critical feature in disease management and referral to a multi-disciplinary pain clinic is required in selected cases.

Summary:

IC/PBS/BPS is a common and under diagnosed condition. A high index of clinical suspicion is required for correct diagnosis and the condition has significant overlap with chronic prostatitis/chronic pelvic pain syndrome in men. Diagnosis can be purely clinical using questionnaires but cystoscopic confirmation and assessment is supportive and can help "personalize" patient treatment. A variety of oral and intravesical agents are available and utilization of a multi-modality treatment approach results in meaningful symptomatic improvement in a large number of patients.

References:

1. Weiss JM. Pelvic floor myofascial trigger points: manual therapy for interstitial cystitis and the urgency-frequency syndrome. J Urol; 166:2226-2231, 2001
2. Nordling J. Primary evaluation of patients suspected of having interstitial cystitis (IC). Eur Urol; 45(5):662-9, 2004
3. Wyndaele JJ. Evaluation of patients with painful bladder syndrome/interstitial cystitis. Scientific World Journal; 5:942-9, 2005
4. Dimitriakov J et al: Pharmacologic management of painful bladder syndrome/interstitial cystitis: a systematic review. Arch Intern Med; 167(18):1922-9, 2007
5. Theoharides TC: Treatment approaches for painful bladder syndrome/interstitial cystitis. Drugs; 67(2):215-35, 2007
6. Evans RJ, Sant GR: Current diagnosis of interstitial cystitis: an evolving paradigm. Urology; 69(4 Suppl):64-72, 2007
7. Sant GR et al: The mast cell in interstitial cystitis: role in pathophysiology and pathogenesis.Urology; 69(4 Suppl):34-40, 2007

Presented by: Grannum R. Sant, MD, FRCS, FACS, at the Masters in Urology Meeting - July 31, 2008 - August 2, 2008, Elbow Beach Resort, Bermuda

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