AUA: Interstitial Cystitis & Bladder Pain Syndrome (2022)

See Original Guidelines

Definition

• Definition of Interstitial Cystitis/Bladder Pain Syndrome (4):
  1. Unpleasant sensation (pain, pressure, discomfort)
  2. Perceived to be related to the urinary bladder
  3. Associated with lower urinary tract symptoms of >6 weeks duration
  4. In the absence of infection or other identifiable causes

Epidemiology

• Studies to define its prevalence are difficult to conduct since there is no objective marker to establish the presence of IC/BPS
• Population-based prevalence studies of IC/BPS have used three methods:
  • Surveys that ask participants if they have ever been diagnosed with the condition (self-report studies)
    • In NHANES III, the self-reported prevalence was 470 per 100,000 population, including 60 per 100,000 men and 850 per 100,000 women.
  • Questionnaires administered to identify the presence of symptoms that are suggestive of IC/BPS (symptom assessments)
    • In the US Nurses Health Study (NHS), questions about IC/BPS symptoms were included and the prevalence of IC/BPS symptoms was 2.3%.
  • Administrative billing data used to identify the number of individuals in a population who have been diagnosed with IC/BPS (clinician diagnosis).
    • Using administrative billing data from the Kaiser Permanente Northwest managed care population in the Portland, Oregon metropolitan area, the prevalence of the diagnosis was found to be 197 per 100,000 women and 41 per 100,000 men.
• May be greatly under-diagnosed in the male population
• Most commonly diagnosed in  individuals over 40, although the diagnosis may be delayed

Natural History

• Psychosocial functioning and QoL consequences of IC/BPS
  • Damaging work life, psychological well-being, personal relationships and general health
  • QoL is poorer
  • Rates of depression are also higher
  • Significantly more pain, sleep dysfunction, catastrophizing, depression, anxiety, stress, social functioning difficulties and sexual dysfunction
• The impact of IC/BPS on QoL is as severe as that of rheumatoid arthritis and end- stage renal disease.

Diagnosis and Evaluation

• The diagnosis of IC/BPS can be challenging. Patients present with a wide spectrum of symptoms, physical exam findings, and clinical test responses.

UrologySchool.com Summary

• Mandatory
  • History and Physical Exam
    • Including baseline voiding symptoms and pain levels
  • Labs
    • Urinalysis +/- culture
• Optional
  • Cystoscopy
  • Urodynamics

Mandatory

History and Physical Exam

• History
  • Signs and Symptoms
    • Pain (including sensations of pressure and discomfort) is the hallmark symptom of IC/BPS.
      • Typical IC/BPS patients report not only suprapubic pain (or pressure, discomfort) related to bladder filling but pain throughout the pelvis, including the urethra, vulva, vagina, rectum, as well in extragenital locations such as the lower abdomen and back
        • Men with IC/BPS is less likely to report perineal pain as their most bothersome symptom. Instead, men with IC/BPS are more likely to have suprapubic tenderness.
      • Many patients use other words to describe symptoms, especially “pressure” and may actually deny pain
      • A key characteristics of pain related to IC/BPS is that the pain is worsened with bladder filling (“painful bladder filling”) and/or their strong urge to urinate was due to pain, pressure, or discomfort (“painful urgency”).
    • May also present with marked urinary urgency and frequency
      • Typically IC/BPS patients void to avoid or to relieve pain; OAB patients, however, void to avoid incontinence.
    • Symptom duration
      • IC is a chronic disorder and symptoms should be present for at least six weeks with documented negative urine cultures for infection
      • Initially it is not uncommon for patients to report a single symptom such as dysuria, frequency, or pain, with subsequent progression to multiple symptoms
    • Symptom flares, during which symptoms suddenly intensify for several hours, days, or weeks, are not uncommon.
    • The number of voids per day, sensation of constant urge to void, and the location, character and severity of pain, pressure or discomfort should be documented. Dyspareunia, dysuria, ejaculatory pain in men, and the relationship of pain to menstruation in women should also be noted.
    • Baseline voiding symptoms and pain levels should be obtained in order to measure subsequent treatment effects.
      • Validated questionnaires such as the GUPI or the ICSI are useful to gather comprehensive symptom information, including symptoms in addition to those of pain or discomfort
  • Past medical history
    • Common for IC/BPS to coexist with other unexplained medical conditions such as fibromyalgia, (IBS), chronic fatigue syndrome, Sjogren's syndrome, chronic headaches, and vulvodynia
    • Patients with IC/BPS frequently exhibit mental health disorders such as depression and anxiety
  • Past surgical history
    • High rate of prior pelvic surgery (especially hysterectomy) and levator ani pain in women with IC/BPS, suggesting that trauma or other local factors may contribute to symptoms
      • the high incidence of other procedures such as hysterectomy or laparoscopy may be the result of a missed diagnosis and does not necessarily indicate that the surgical procedure itself is a contributing factor to symptoms

• Physical Exam
  • Abdomen
    • Suprapubic tenderness is common
    • Presence of hernias
  • Pelvis
    • Palpation of the external genitalia, bladder base in females, and urethra in both sexes.
    • The pelvic floor muscles in both sexes should be palpated for locations of tenderness and trigger points.
    • The pelvic support for the bladder, urethra, vagina, and rectum should be documented.
    • A focused evaluation to rule out vaginitis, urethritis, tender prostate, urethral diverticulum, or other potential sources of pain or infection is important.
  • Neurologic
    • A brief neurological exam to rule out an occult neurologic problem and an evaluation for incomplete bladder emptying to rule out occult retention should be done on all patients.

Labs

Urinalysis +/- culture

• Urine culture may be indicated even in patients with a negative urinalysis in order to detect lower levels of bacteria that are clinically significant but not readily identifiable with a dipstick or on microscopic exam

Optional

• Cystoscopy and/or urodynamics should be considered when the diagnosis is in doubt; these tests are not necessary for making the diagnosis in uncomplicated presentations.

Cystoscopy

• Cystoscopy with hydraulic distention of the bladder in men with IC/BPS commonly demonstrates diffuse glomerulations
• Hunner lesions can be identified on cystoscopy in men with IC/BPS.
• Indications
  • Excluding conditions that may mimic IC/BPS (bladder cancer, bladder stones, urethral diverticula, and intravesical foreign bodies)
  • Identification of a Hunner lesions
    • Hunner lesions
      • Common in IC/BPS patients of age over 50 years.
      • The only consistent cystoscopic finding that leads to a diagnosis of IC/BPS, though there are no agreed-upon cystoscopic findings diagnostic for IC/BPS
      • May be identified in an acute phase (as an inflamed, friable, denuded area) or a more chronic phase (blanched, nonbleeding area)
• Bladder biopsy may be indicated to exclude other pathologies if a lesion of uncertain nature is present but is not part of the routine diagnostic process and presents a risk of perforation.
• Cystoscopy with hydrodistension under anesthesia
  • The finding of glomerulations on hydrodistention (less than 80 cm H2O, less than 5 minutes) is variable and not consistent with clinical presentation
  • Glomerulations (pinpoint petechial hemorrhages) may be detected on cystoscopy but these lesions are non-diagnostic and non-specific for IC/BPS and are commonly seen in other conditions which may co-exist with or be misdiagnosed as IC/BPS such as chronic undifferentiated pelvic pain or endometriosis.
  • Glomerulations may also be present in asymptomatic patients undergoing cystoscopy for other conditions.
  • glomerulations may be seen in patients who have undergone radiation therapy, in the presence of active bladder carcinoma, associated with chemotherapeutic or toxic drug exposure, and in patients with defunctionalized bladders, and in patients without any urologic symptoms.
  • Hydrodistension is not necessary for routine clinical use to establish a diagnosis of IC/BPS diagnosis.
    • If hydrodistension is performed to determine whether Hunner lesions are present or as a treatment, then the technique should be specified and the bladder capacity determined. It is useful for the clinician and patient to understand when bladder capacity is severely reduced (a low capacity due to fibrosis).

Urodynamics

• No agreed-upon urodynamic criteria diagnostic for IC/BPS
• Pain with filling (hypersensitivity) is consistent with IC/BPS.
• Not recommended for routine clinical use to establish an IC/BPS diagnosis.
• Indications
  • Suspicion of outlet obstruction in either sex
  • Possibility of poor detrusor contractility
  • Other conditions that could explain why patients are initially refractory to first-line therapy

Not recommended

• Potassium sensitivity test
  • Might help to identify the patients who are most likely to respond to urothelium-restoring treatments
  • Risk/benefit ratio was too high for routine clinical use

Differential Diagnosis

1. Bacterial cystitis
2. Urinary calculi
3. Vaginitis
4. Carcinoma in situ of the bladder
5. Chronic bacterial prostatitis

Management

• IC/BPS is a heterogeneous clinical syndrome.
  • Some IC/BPS patients have bladder-centric phenotypes (e.g., Hunner lesions, small bladder capacity, pain improved with intravesical local anesthetics). Patients with Hunner lesions should be treated differently from those without Hunner lesions (Statement 19 and Algorithm). Others have pelvic-floor phenotype (e.g., pelvic floor tenderness on exam). Women with this feature respond better to pelvic floor manual physical therapy (Statement 12). A third group have systematic or widespread symptoms characterized by the presence of “widespread pain”26 (significant non-urologic pain outside the pelvis), COPC (e.g., fibromyalgia, IBS),25 widespread psychosocial difficulties (e.g., anxiety, depression, higher levels of current and lifetime stress, early life and adult traumatic events, negative affect, poor illness coping),36 or poly-symptomatic, poly-syndromic(PSPS) presentation with widespread somatic symptoms across multiple organ systems.
• Response to therapy is associated with improved overall QoL.48 In addition, response to therapy is associated with improved sexual function and sleep, with concomitant improvements in QoL
• A trial of antibiotic therapy is appropriate when infection is suspected; if symptoms resolve a course of antibiotic suppression may be considered to allow for full recovery.