In 1987, for research purposes, the National Institutes of Health (NIH) established standardized diagnostic criteria for IC (Box 29-1). The National Interstitial Cystitis Data Base (ICDB), a cooperative multicenter longitudinal observation study group established in 1991, was sponsored to study the natural history of the disease and was based on a large population with symptoms consistent with IC. In 1999 the ICDB reported that the NIH guidelines were too restrictive to be used by clinicians. If the initial criteria were used, up to 60% of symptomatic women would not be diagnosed. Following an International Consensus in Kyoto, Japan, in April, 2003, the NIH assigned a panel to update the definition and management of IC. The guidelines are pending.

BOX 29-1 NIH-NIDDK DIAGNOSTIC CRITERIA OF INTERSTITIAL CYSTITIS

Category A: At least one of the following findings on cystoscopy:

• Diffuse glomerulations (at least 10 per quadrant) in at least 3 quadrants of the bladder

• A classic Hunner’s ulcer

Category B: At least one of the following symptoms:

• Pain associated with the bladder

• Urinary urgency

Exclusion criteria:

• Age <18 years ^*

• Urinary frequency while awake <8 times per day

• Nocturia fewer than two times per night

• Maximal bladder capacity >350 mL while patient is awake

• Absence of an intense urge to void with bladder filled to 150 mL of water with medium filling rate (30–100 mL/min) during cystometry

• Involuntary bladder contractions on cystometry using medium filling rate

• Duration of symptoms <9 months ^*

• Symptoms relieved by antimicrobial agents (antibiotics, urinary antiseptics), anticholinergics, or antispasmodics ^*

• Urinary tract or prostatic infection in the past 3 months ^*

• Active genital herpes

• Vaginitis ^*

• Uterine, cervical, vaginal, or urethral cancer within the past 5 years ^*

• Bladder or ureteral calculi ^*

• Urethral diverticulum ^*

• History of cyclophosphamide or chemical cystitis or tuberculosis or radiation cystitis

• Benign or malignant bladder tumors

NOTE: These are the original criteria and are changing.

^* Relative exclusion criteria.

Gillenwater JY, Wein AJ. Summary of the National Institute of Arthritis, Diabetes, Digestive and Kidney Diseases Workshop on Interstitial Cystitis, National Institutes of Health. Bethesda, MD, August 28–29, 1987. J Urol 1988;140:203.

ETIOLOGY

The etiology of IC is currently unknown. Most authors believe it is multifactorial. Currently proposed causes include infectious agents, quantitative glycosaminoglycan (GAG) layer deficiency, ultrastructural abnormality of the lamina propria or interstitium of the bladder, mast cells, and neurogenic inflammation. The development and complete characterization of a promising urinary marker, antiproliferative factor (APF) may aid in our understanding of the etiology, diagnosis, and management of IC.

INFECTIOUS AGENTS

Extensive efforts have been made, with limited success, to establish an infectious agent as the cause of IC. Hunner (1915) first suggested a hematogenously disseminated bacterial cystitis as the cause. Most patients with IC report a history of UTI and have received several courses of antibiotics based on their symptoms, not on positive urine cultures. To date, no single bacterium, virus, fungus, or fastidious microorganism has been isolated as an etiologic factor in IC.

Some authors believe that a low bacterial count, bacterial antigens, or by-products, such as endotoxins or P-fimbriae, may be involved. These substances can activate an autoimmune response or cause sensory nerve stimulation, thus activating the neurogenic inflammation model of IC. DNA sequencing of bladder biopsies searching for bacteria or their by-products has demonstrated controversial results.

Diagnosis

Painful bladder syndrome is a diagnosis of exclusion (see Box 29-1). Patients cannot have evidence of cystitis caused by infection, use of cyclophosphamide or other chemical agents, radiation, or tuberculosis. Other infections, such as vaginitis, urethritis, urethral Ureaplasma, or genital herpes, cannot be present. Also, urethral diverticulum, bladder carcinoma, and carcinoma-in-situ must be excluded. However, patients with painful bladder syndrome may have concomitant lower urinary tract or pelvic floor disorders.

HISTORY

Frequency (greater than eight voids during waking hours), nocturia (greater than two voids during sleeping hours), urgency, suprapubic pressure on bladder filling, bladder pain, and dyspareunia are the most common symptoms. Secondary symptoms include burning during or after urination; a feeling of decreased bladder emptying; hesitancy; interruption of urinary stream; double voiding; referred pain to the abdomen, lower back, or medial thighs; abdominal bloating; postvoid urethral pain; incontinence without sensation; or detrusor overactivity. The severity and duration of each flare over the previous year should be recorded. The physician should also inquire about previous UTI, types of incontinence, parity, previous pelvic surgery, radiation therapy, chemotherapy, previous treatments, menstrual history, history of endometriosis, central nervous system trauma, and dyspareunia. Commonly, patients initially present with one symptom only, urinary urgency/frequency, nocturia, or pain. All patients should be asked to do a voiding diary (see Fig. 14-1) and fill out the O’Leary-Sant symptom and problem index (Fig. 29-3). The recently validated pelvic pain urgency and frequency (PUF) questionnaire may also be used. A medical history should include associated diseases, such as migraines, fibromyalgia, sinusitis, drug hypersensitivity, sicca syndrome (dry eyes, dry mouth), Sjögren’s syndrome, vulvodynia, and irritable bowel syndrome.

Figure 29-3 O’Leary-Sant Interstitial Cystitis Symptom Index.

PHYSICAL EXAMINATION

The abdominal and back examinations detect the presence of costovertebral tenderness and abdominal tenderness or mass. The external genitalia are examined for signs of infection and vulvar vestibulitis (tenderness over the vulvar vestibular or Bartholin’s glands). The pelvic examination includes a vaginal and urethral culture, if appropriate, evaluation for signs of atrophic vaginitis or a urethral caruncle, and a Pap smear if indicated. Careful palpation and perineometry may help evaluate the pelvic floor muscles to rule out pelvic floor dysfunction (the inability to optimally contract and relax the pelvic floor muscles). Evaluation of bladder and pelvic organ support is done in the dorsal lithotomy and standing positions to determine and grade bladder neck hypermobility, cystocele, enterocele, uterine prolapse, rectocele, and perineal descent. The posterior bladder wall and urethra should be palpated to check for tenderness and masses, and a bimanual examination should be performed to detect pelvic or adnexal masses and tender nodules. A rectovaginal examination is performed to evaluate tenderness along the uterosacral ligaments. Finally, a neurourologic examination ascertains the presence of the bulbocavernosus reflex and grades perineal sensation and anal sphincter strength.

LABORATORY AND RADIOGRAPHIC EVALUATION

Initial laboratory examination should include urinalysis, urine culture and sensitivity, and measurement of postvoid residual urine volume (catheterized or with bladder ultrasound). Urine cytology is indicated in the presence of hematuria and persistent urgency. A 24-hour voiding diary measures input and output, number and quantity of voids, and number and severity of leakage episodes. A renal ultrasound, intravenous pyelogram (IVP), or computed tomography (CT) scan is indicated in the presence of hematuria, history of recurrent UTI, or history of pelvic surgery. A voiding cystourethrogram (VCUG) is useful in ruling out vesicoureteral reflux and evaluating the bladder neck in patients with concomitant incontinence or suburethral diverticulum. Magnetic resonance imaging (MRI) may further delineate the urethra when evaluating for a urethral diverticulum. Awake cystoscopy is indicated in the presence of hematuria, persistent or recurrent UTI, or suspected fistula or urethral diverticulum.

POTASSIUM SENSITIVITY TEST

Parsons (1996) designed the potassium sensitivity test (PST) to measure epithelial permeability. Instilling a solution of potassium chloride (KCl) into a normal bladder will not promote urgency or pain (Box 29-2). If a patient’s bladder has a leaky epithelium, the KCl mixture readily diffuses across the transitional cells and stimulates sensory nerves, causing urgency and pain. Parsons demonstrated that 70% of patients with IC had provocation of symptoms, whereas only 4% of normal subjects responded with pain. A recent study showed that 84% of patients with a score of >14 of the PUF questionnaire had a positive PST.

BOX 29-2 POTASSIUM SENSITIVITY TEST

Measure of epithelial permeability: KCl solution (40 mL; 400 mEq/L)

Normal bladder intact epithelium: KCl provokes no symptoms

Epithelial dysfunction: K⁺ diffuses across transitional cells and stimulates sensory nerves, causing urgency or pain

Positive response: no response to H₂O; ≥2 response to KCl (scale 0–5)

CYSTOSCOPY

Cystoscopy is both diagnostic and therapeutic. Cystoscopy with hydrodistention of the bladder traditionally has been performed under general or regional anesthesia. Our experience has been that intravenous sedation, bupivacaine hydrochloride (Marcaine) bladder instillation, and pudendal nerve block are effective diagnostic and therapeutic alternatives with minimal morbidity and increased postoperative patient satisfaction. Cystoscopy is performed to eliminate other causes of persistent patient symptoms and hematuria and to examine for typical findings of IC (Table 29-1). The classic Hunner’s ulcer, an area of erythema with small vessels radiating to a central, pale scar after bladder filling, is found in only 8% of patients with IC. Previous biopsy sites and carcinoma in situ can be confused with ulcers. Before hydrodistention, an inclusive evaluation of the bladder is performed, and the degree of hypervascularity and linear scarring (mild, moderate, severe) is assessed. Hydrodistention is performed by filling the bladder under gravity at 80 cm H₂O with urethral compression for 2 to 7 minutes. The bladder is then drained, looking for a terminal bloody effluent, and the capacity is measured (>350 mL is defined as early-stage IC and ≤350 mL as late-stage IC). The average bladder capacity for patients with IC has been reported as 575 mL and as 1115 mL for patients without persistent irritative voiding symptoms. On redistention of the bladder, the presence and severity of glomerulations, strawberry-like petechial hemorrhages, are determined. Not all patients with significant symptoms have glomerulations or a reduced bladder capacity. Bladder biopsy is performed after hydrodistention to reduce the risk of perforation. The biopsy eliminates the presence of carcinoma in situ and infection, and it can quantitate the number of mast cells in the lamina propria and detrusor muscle. The diagnosis of IC cannot be ruled out based on normal cystoscopic findings; the findings of glomerulations and a terminal bloody effluent suggest the diagnosis of IC. A capacity below 350 mL or the presence of a Hunner’s ulcer confirms the diagnosis of late IC. Concomitant laparoscopy may be performed when there is tenderness in the adnexa, a history of previous pelvic surgery, infertility, an abnormal pelvic ultrasound, suspected endometriosis, dysmenorrhea, or dyspareunia in the absence of vulvodynia.

Table 29-1

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