Inclusion criteria
1. Cystoscopy – glomerulations and/or classic Hunner’s ulcer
2. Symptoms – bladder pain and/or bladder urgency
Exclusion criteria
1. Bladder capacity greater than 350 cc on awake cystometry
2. Absence of an intense urge to void with the bladder filled to 100 cc during cystometry using a fill rate of 30–100 cc/min
3. Demonstration of phasic involuntary bladder contractions on cystometry using the fill rate described in number 2
4. Duration of symptoms less than 9 months
5. Absence of nocturia
6. Symptoms relieved by antimicrobials, urinary antiseptics, anticholinergics, or antispasmodics
7. Frequency of urination while awake of less than eight times a day
8. Diagnosis of bacterial cystitis or prostatitis within a 3-month period
9. Bladder or ureteral calculi
10. Active genital herpes
11. Uterine, cervical, vaginal, or urethral cancer
12. Urethral diverticulum
13. Cyclophosphamide or any type of chemical cystitis
14. Tuberculous cystitis
15. Radiation cystitis
16. Benign or malignant bladder tumors
17. Vaginitis
18. Age less than 18 years
The definition proposed by the standardization subcommittee of the International Continence Society [1] (2012) states the condition as “the complaint of suprapubic pain related to bladder filling, accompanied by increased day time and night time urinary frequency in the absence of proven urinary tract infection or other pathology” and suggested the term IC be restricted to specific cystoscopic and histological features [9] (the features were however not defined). The condition came to be known as PBS/IC or IC/PBS. The diagnostic sensitivity for PBS using these criteria was only 61 % [10]. At the clinical proceedings of the Association of Reproductive Health Professionals in 2007, the definition of PBS/IC was proposed as: “Pelvic pain, pressure or discomfort related to the bladder, typically associated with persistent urge to void or urinary frequency, in the absence of urinary infection or other pathology” [11].
Revisions have been proposed to the terminology by several clinicians and organizations interested in clarifying this issue. This includes the NIDDK Research Symposium: Frontiers in Painful Bladder Syndrome and Interstitial Cystitis in 2006 proposing the terminology bladder pain syndrome (BPS), the NIDDK Multidisciplinary Approach to the Study of Chronic Pelvic Pain Network (2008), and the American Urological Association (AUA) IC/BPS Guideline 2011 attempting to redefine it [12].
The European Association of Urology (EAU) 2012 Guidelines on Chronic Pelvic Pain and the updated International Association for the study of Pain (IASP) Taxonomy 2012 defines BPS as follows: “Bladder pain syndrome is the occurrence of persistent or recurrent pain perceived in the urinary bladder region, accompanied by at least one other symptom, such as pain worsening with bladder filling, daytime and/or night-time urinary frequency. There is no proven infection or other obvious local pathology.” Bladder pain syndrome is often associated with negative cognitive, behavioral, sexual, or emotional consequences as well as with symptoms suggestive of lower urinary tract and sexual dysfunction [1, 13]. The European Society for the Study of Interstitial Cystitis (ESSIC) proposed that the definition incorporate features: “Chronic (6 months or more) pelvic pain, pressure or discomfort perceived to be related to the urinary bladder accompanied by at least one other urinary symptom like persistent urge to void or urinary frequency. Confusable diseases as the cause of the symptoms must be excluded” [14].
Risk Factors/Etiology
The etiology of BPS is unknown but there are several hypotheses regarding its pathophysiology. A series of events have been proposed to trigger the BPS such as bladder overdistension, bacterial infections, trauma from pelvic surgery, and bladder trauma, which are believed to damage the bladder epithelium [11] (Fig. 11.1).
Fig. 11.1
Proposed pathophysiology of BPS
Dysfunction of the bladder epithelium makes the coating of the bladder epithelium, the glycosaminoglycan (GAG) layer, to become defective. This can increase the permeability of bladder epithelium to urine contents such as potassium, which can leak into the interstitium and musculature, leading to mast cell activation and release of histamine. It is also supposed to activate the C-fiber nerve endings and release of substance P. Immunologic and allergic responses are postulated to be triggered as well. Combination of these events is believed to cause progressive bladder damage and chronic neuropathic type of pain [15].
Associated Conditions
Hypersensitivity disorders like irritable bowel syndrome, fibromyalgia, vulvodynia, myofascial dysfunction of the pelvic floor muscles, migraine headaches, and allergies are more common among patients with symptoms of BPS compared to the general population [16].
Clinical Features
Symptom presentation in BPS is variable, usually presenting with pelvic pain or bladder pain, pressure or discomfort relating to bladder, urinary urgency, and urinary frequency. Pain is typically chronic in nature (>6 months), referred in the area of the bladder, vagina, urethra, rectum, or perineum. The characteristic feature of the pain is its relation to bladder filling, increasing with bladder distension which may diminish with voiding [16]. In some patients the pain may persist for a considerable time after voiding, with a persistent urge to void. Urinary frequency is common with BPS and usually occurs both at daytime and night time. Urgency is a feature common to both BPS and overactive bladder (OAB) and is one of the reasons for misdiagnosis of this condition. Urgency in OAB is typically due to concern about an impending incontinence, whereas in BPS it is due to increasing pain with filling and an attempt to relieve it by voiding.
There are no specific features for BPS on physical examination, but most often patients with BPS have bladder base tenderness on pelvic examination and tenderness of levator ani muscle has been noted in 81 % of patients in a study [17]. Pain mapping of the vulva can be done to rule out vulvar/vestibular diseases.
Investigations
The diagnosis of BPS is one of exclusion, and hence assessment of these patients should aim to rule out other conditions which can present with similar features, such as urinary tract infection, endometriosis, bladder carcinoma, radiation cystitis, overactive bladder, and nonbacterial cystitis secondary to drugs such as NSAID, allopurinol, aspirin, and cyclophosphamide. It is important to remember that some of these conditions can be associated with BPS and a diagnosis of BPS is possible only if symptoms persist after treatment of the associated conditions.
Evaluation, along with a detailed history and examination should include a 3-day bladder diary, which can provide information about the frequency and volume of voids. Urine analysis and culture are needed to rule out bacterial cystitis and atypical infections like chlamydia or mycoplasma. In the presence of a hematuria, urine cytology is indicated to rule out bladder carcinoma.
Urodynamics
The role of urodynamics is to rule out detrusor overactivity and there are no specific features diagnostic of BPS. However in patients with BPS, on uroflowmetry, the volume voided is smaller compared to those with idiopathic detrusor activity. In addition on filling CMG, values for bladder sensation such as first desire to void, normal desire to void, and strong desire to void are significantly lower in patients with BPS due to increased bladder sensation. Pain on increased filling also means bladder may not be increased to its capacity leading to decreased bladder compliance [18].
Cystoscopy
In the past, cystoscopy was primarily employed to identify the Hunner’s ulcer, a primary diagnostic criteria in the NIDDK criteria. Currently its role is to exclude intravesical abnormalities in the bladder such as endometriosis, carcinoma, or foreign body. In the presence of mucosal ulcers, glomerulations, or edema on cystoscopy, the symptoms appear to be severe in nature.
Potassium Sensitivity Testing (Parsons Test)
The test involves instillation of 40 ml of sterile water and 40 ml of 40 mEq/100 ml potassium chloride alternatively into the bladder for 5 min. Pain and urgency following each instillation are noted. Increase in pain or urgency with potassium instillation indicates a positive test. The test is painful, has low sensitivity and specificity, and hence is not used anymore.
Urine Markers
Antiproliferative factor, epidermal growth factor, insulin-like growth factor (IGF)-binding protein 3, and interleukin (IL)-6 are noted to be increased in patients with interstitial cystitis, whereas markers such as cyclic GMP and methyl histamine are decreased in these patients [19]. Among these, the antiproliferative factor shows promise as marker for BPS in the future.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
