Interstitial Cystitis Clinical Presentation

Updated: Nov 14, 2018
  • Author: Eric S Rovner, MD; Chief Editor: Edward David Kim, MD, FACS  more...
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Presentation

History

Because interstitial cystitis is a poorly defined entity of unknown etiology, the clinical presentation is often not uniform and the symptoms vary in severity and nature. The onset of symptoms is often, but not invariably, acute, and the patient is sometimes able to describe the moment at which symptoms began. Patients often associate the onset of symptoms with a specific urinary tract infection (UTI), catheterization, or bladder or pelvic surgery.

Symptoms of interstitial cystitis may include the following:

  • Urinary frequency, urgency, and pain
  • A sense of pressure, discomfort, or pain in the pelvis
  • A vague sense of incomplete bladder emptying
  • A constant sensation of needing to void, or a compulsion to void
  • Dyspareunia, sex-related distress, and decline in libido and orgasm frequency are also common

Interstitial cystitis is characterized by periods of exacerbation followed by variable periods of remission; some patients have completely asymptomatic periods interspersed with flares. Symptoms may vary daily or weekly or may be constant and unrelenting for months or years and then resolve spontaneously with or without therapy. Spontaneous remission occurs in as many as 50% of patients at a mean of 8 months. In females, symptoms may fluctuate relative to the ovulatory cycle. As with other autoimmune conditions, some pregnant women may experience periods of remission during the second and third trimester, further supporting an immune-mediated pathophysiology.

The most prevalent feature of interstitial cystitis is irritative lower urinary tract symptoms, including urinary frequency in association with varying degrees of pain. The exact number of micturitions, daytime or nighttime, is not important; however, according to the National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK) criteria, more than 8 micturitions per day is considered adequate for inclusion into clinical studies. Daytime frequency in the absence of nocturia is not characteristic of interstitial cystitis. The absence of significant nocturia may suggest an alternative diagnosis (eg, sensory urgency). Urinary incontinence is quite rare. Patients with a primary complaint of incontinence may require further evaluation, including urodynamic studies.

Pain with bladder filling is a common finding that may be reproduced urodynamically or with cystography. Patients may report constant pelvic pain or pain related to a full bladder. Such pain is often relieved partially or wholly by voiding. Dysuria (pain with voiding) may be associated with interstitial cystitis; however, this is a distinct symptom that may imply another diagnosis and suggests that additional evaluation of the lower urinary tract and genital tract is indicated. Dyspareunia is common in as many as 50% of women.

Men with interstitial cystitis may report perineal, groin, penile, or scrotal pain.The diagnosis of prostadynia or nonbacterial prostatitis (chronic pelvic pain syndrome) should be entertained in these patients.

Patients with interstitial cystitis have a high incidence of associated conditions, including allergies, irritable bowel syndrome, fibromyalgia, and focal vulvitis. A substantial emotional and psychological overlay to the complaints, due to the duration and severity of symptoms, may or may not be present.

In a population-based sample of 3397 women with interstitial cystitis/bladder pain syndrome (IC/BPS), more than half reported poor sleep quality, sleep duration of 6 hours or less, or trouble sleeping due to symptoms. Short sleep duration was significantly associated with greater impairment in IC/BPS quality of life and poorer self-reported physical health. Poor sleep quality was significantly associated with greater quality of life impairment, poorer physical health, and increased sexual dysfunction. Nocturnal IC/BPS symptoms were significantly associated with greater IC/BPS impairment, poorer physical and mental health, and greater sexual dysfunction. [42]

Questionnaires

Validated questionnaires may serve as an aid in clinical diagnosis, as a means of tracking symptom response to therapy in clinical practice, or as an assessment of response to treatments in study populations. However, despite the usefulness of these metrics, interstitial cystitis remains a diagnosis of exclusion. The Wisconsin Interstitial Cystitis Scale was initially validated in a small population [43] and has subsequently been shown in larger studies to be valid and easy to implement. [44] It has also been shown to correlate well with other validated interstitial cystitis questionnaires. [45]

The Interstitial Cystitis Symptom Index and the Problem Index (O'Leary-Sant Interstitial Cystitis Symptom Index and Problem Index) are self-administered questionnaires that have been found to be valid and reliable and to serve as useful adjuncts to aid in diagnosis. They were not designed to be used as screening tools. [46] Theses indices have also been shown to be responsive to changes in interstitial cystitis symptoms [47] and may therefore be useful in measuring response to therapy in clinical and research settings.

The Pelvic Pain and Urgency/Frequency Patient Symptom Scale is a tool that can be used for screening, and it measures both urinary symptoms, such as pain and urgency, and symptoms related to sexual intercourse. It has 2 scales, one that measures symptoms and a second "bother score" that measures how troublesome these symptoms actually are to the patient. Parsons et al showed that this scale identified accurately 74% of patients with IC/BPS with scores of greater than 10 and 91% of patients with scores of 20 or greater. Additionally, it is easily filled out and has been used as a monitoring tool to assess treatment response. [48]

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Physical Examination

Abdominal, pelvic, and directed neurologic examinations should be performed in all patients with voiding dysfunction. Nevertheless, the findings from these examinations are often unrevealing in patients with interstitial cystitis. Women with interstitial cystitis may express some discomfort with palpation over the urethra and bladder base. A correlation has been noted between urethral tenderness and the finding of a Hunner ulcer on cystoscopic examination.

Pain upon urethral palpation in the presence of an anterior vaginal wall mass may suggest urethral diverticulum, whereas cervical motion tenderness may suggest pelvic inflammatory disease. On examination with a speculum, any of the following findings suggest a diagnosis other than interstitial cystitis:

  • Prolapse
  • Masses
  • Evidence of vaginitis, herpes, vestibular adenitis, vulvovestibulitis, vulvodynia, or other pathology

Palpation for a full bladder and bimanual examination evaluating for adnexal masses should be part of the complete examination. Rectal examination should always be performed to evaluate for masses or tenderness and to assess rectal and pelvic floor (levator) muscle tone.Neurologic examination findings are usually unremarkable, but abnormalities of motor function, sensation, or reflexes may indicate spinal cord or nerve root dysfunction and should prompt further evaluation for other diagnoses.

Physical examination findings can help differentiate interstitial cystitis/bladder pain syndrome from provoked vestibulodynia disorder (PVD).  To examine the vestibule (the tissue surrounding the urethral meatus, at the opening of the vagina), the clinician moves the labia minora to the side and lightly touches the tissue with a cotton swab. This maneuver is normally painless, but can elicit burning or cutting pain or pelvic pain in a woman with PVD. [49]

Pain on vestibular examination should prompt investigation of the cause of PVD (eg, testosterone deficiency, neurologic factors, pelvic muscle hypertonicity), as this will determine treatment. Testosterone deficiency is treated with cessation of oral contraceptives or, in menopausal women, application of estrogen/testosterone creams. Neuroproliferative PVD is treated with vulvar vestibulectomy. Hypertonicity of the pelvic floor muscles is treated in part with physical therapy. [49]

Male patients commonly have no abnormalities upon examination. In male patients with irritative lower urinary tract symptoms, bladder outlet obstruction and chronic nonbacterial prostatitis are important diagnostic considerations.

NIDDK criteria

Because no pathognomonic criteria exist for the diagnosis of interstitial cystitis, the modified National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK) criteria for the inclusion of patients in interstitial cystitis basic and clinical research studies can be used. These criteria, initially developed in 1987, were originally intended not to define interstitial cystitis but to ensure that studies of the disease had relatively comparable patient populations. Nevertheless, the criteria became the de facto definition of the disease, although a significant number of patients with interstitial cystitis do not meet the criteria.

The NIDDK criteria for interstitial cystitis include 2 sets of inclusion criteria and 1 of exclusion criteria. For the inclusion criteria, 1 of the cystoscopic findings and at least 1 subjective symptom must be present. All of the exclusion criteria must be absent.

Cystoscopic criteria include 1 of the following findings after distention under anesthesia for 1-2 minutes at 80-100 cm water (bladder must be distended up to 2 times before evaluation):

  • Glomerulations in at least 3 quadrants of the bladder and at least 10 glomerulations per quadrant

  • Classic Hunner ulcer

  • Glomerulations must not be along the path of the cystoscope

Subjective symptoms include the following:

  • Pain associated with the bladder

  • Urinary urgency

The presence of any of the following criteria is intended to exclude patients with other diseases that can cause bladder symptoms and patients with atypical characteristics:

  • Cystometric bladder capacity greater than 350 mL in a conscious patient with either gas or liquid filling

  • Absence of an intense urge to void when patient's bladder has been filled with 100 mL of gas or 150 mL water during cystometry at a fill rate of 30-100 mL/min

  • Demonstration of phasic involuntary bladder contractions on cystometry findings at a fill rate of 30-100 mL/min (note that although this is an exclusion criterion per the NIDDK, detrusor instability may be present in as many as 14% of patients with a clinical diagnosis of interstitial cystitis)

  • Duration of symptoms less than 9 months

  • Nocturia

  • Symptoms relieved by antimicrobials, urinary antiseptics, anticholinergics, or antispasmodics

  • Micturition frequency of less than 8 times daily

  • Diagnosis of bacterial prostatitis or cystitis within a 3-month period

  • Presence of ureteral or bladder calculi

  • Active genital herpes

  • Uterine, cervical, vaginal, or urethral cancer

  • Urethral diverticulum

  • Cyclophosphamide or other chemical cystitis

  • Tuberculous cystitis

  • Radiation cystitis

  • Benign or malignant bladder tumors

  • Vaginitis

  • Age younger than 18 years

It should be noted, however, that strict application of these criteria would have misdiagnosed more than 60% of patients who were diagnosed by researchers as definitely or likely having IC/BPS. [50]

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