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Definition

BPS is a chronic condition with unknown aetiology.

The widespread definition for BPS is that proposed by the European Society for the Study of BPS (ESSIC) in 2008 as ‘pelvic pain, pressure or discomfort perceived to be related to the bladder, lasting at least 6 months, and accompanied by at least one other urinary symptom, for example persistent urge to void or frequency, in the absence of other identifiable causes’.

The American Urological Association has described BPS as ‘an unpleasant sensation (pain, pressure, discomfort) perceived to be related to the urinary bladder, associated with lower urinary tract symptoms of more than 6 weeks duration, in the absence of infection or other identifiable causes’.

The term BPS has been recommended rather than the previous names of interstitial cystitis (IC) and painful bladder syndrome.

Hunner lesions are well – demarcated, reddish, mucosal lesions lacking in the normal capillary structure, which usually bleeds.  Hunner lesions may be seen as inflamed friable areas or non-blanching areas in the chronic state.

In 1987, the National Institute of Diabetes and Digestive and Kidney Diseases, one of the US National Institutes of Health, developed diagnostic criteria for the condition with the following inclusions:

  1. pain associated with bladder or urinary frequency, and
  2. glomerulations (pinpoint petechial haemorrhages) on cystoscopy
  3. classic Hunner lesions seen after hydrodistension under anaesthesia to 80–100 cm water pressure for 1–2 minutes, where the glomerulations must be diffuse and present in at least three quadrants of the bladder at a rate of at least 10 per quadrant and not along the path of the cystoscope as this may be an artefact.

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Management of Bladder Pain Syndrome (BPS)

Initial Clinical Assessment:-

Bladder pain syndrome is a chronic pain syndrome.

Total medical history should be taken and physical examination performed.

As the diagnosis of BPS is one of exclusion, it is important to rule out other possible causes of bladder pain. The history taken should include details of previous pelvic surgery, urinary tract infections (UTIs), sexually transmitted infections, bladder disease and autoimmune disease.

Other conditions commonly associated with BPS, such as irritable bowel syndrome, vulvodynia, endometriosis, fibromyalgia, chronic fatigue syndrome and autoimmune diseases like systemic lupus erythematosus and Sjogren’s syndrome, should be enquired about while taking a clinical history.

Physical examination should rule out the following: –

  1. bladder distension due to urinary retention,
  2. hernias and
  3. painful trigger points on abdominal palpation.

A genital examination should be performed to rule out: –

  1. atrophic changes, prolapse, vaginitis and trigger point tenderness over the urethra, vestibular glands, vulvar skin or bladder.
  2. Features of dermatosis, including vulvar or vestibular disease, should be looked for.
  3. An evaluation of the introitus and tenderness during insertion or opening of the speculum should be made.
  4. Superficial/deep vaginal tenderness and tenderness of the pelvic floor muscles should be assessed during the examination.
  5. Cervical pathology should be excluded.
  6. A bimanual pelvic examination is helpful to rule out abdominal, cervical or adnexal pathology.

Baseline Investigations for Bladder Pain Syndrome (BPS)

A bladder diary (frequency volume chart): – A 3-day fluid diary with input and output is useful for initial assessment.  Patients with BPS classically void small volumes, so this is useful to identify the severity of the storage symptoms.  The first morning void is a useful guide to the functional capacity of the bladder.

Food diary to identify if specific foods cause flare-up of symptoms.

Urine to rule out urinary tract infection  as this is a prerequisite for diagnosis of the BPS – A dipstick should be performed and where there is suggestion of a UTI, a culture and sensitivity test.  Testing for acid – fast bacilli where there is sterile pyuria.

Other more common causes of sterile pyuria that should be considered are urinary tract stones, partially treated UTIs and carcinoma in situ of the bladder.

Ureaplasma is not isolated in routine culture tests, so would need to be specifically looked for.

In symptomatic patients with negative urine cultures and pyuria.

Investigations for urinary ureaplasma and chlamydia.

In those with a suspicion of urinary malignancy, urinary cytology should be tested.

Cystoscopy and referral to urology in accordance with local protocols.

Differential Diagnosis of Bladder Pain Syndrome

  1. Malignancy, e.g., bladder carcinoma/carcinoma in situ, cervical, uterine or ovarian cancer
  2. Infection of the urinary or genital tract
  3. overactive bladder
  4. radiation cystitis or drug-mediated cystitis, e.g., cyclophosphamide, ketamine
  5. bladder outlet obstruction or incomplete bladder emptying
  6. calculus of the bladder or lower ureter
  7. urethral diverticulum
  8. pelvic organ prolapse
  9. endometriosis
  10. pudendal nerve entrapment or pelvic flow muscle – related pain.
  11. Irritable bowel syndrome.
  12. Diverticular disease of the bowel.

Ketamine is used as a recreational drug due to its hallucinogenic effects. Unfortunately, its abuse has led to ketamine cystitis, where the bladder becomes ulcerated and fibrosed with urinary tract symptoms, such as frequency, urgency and haematuria, along with renal impairment.

Cystoscopy for Bladder Pain Syndrome (BPS)

Cystoscopy without hydrodistension is expected to be normal (except for discomfort and reduced bladder capacity) in the majority of patients with BPS.

Characteristic cystoscopic findings of BPS include post distension glomerulations, reduced bladder capacity and bleeding.

Pathological features have been described in patients with BPS, including inflammatory infiltrates, detrusor mastocytosis, granulation tissue and fibrosis, but these are nonspecific.

Bladder biopsy may be used to classify BPS or may be indicated to exclude other pathologies, such as carcinoma in situ, if suspected by a focal lesion or abnormal cytology. Hunner lesions are present in type 3 BPS and can be associated with reduced bladder capacity.

Caution should be exercised as there is the recognised risk of bladder perforation and rupture associated with cystoscopy and hydrodistension.

Urodynamic Tests for Bladder Pain Syndrome (BPS)

During urodynamic studies, pain on bladder filling, a reduced first sensation to void and reduced bladder capacity are consistent with BPS; however, there are no urodynamic criteria that are diagnostic for BPS.

Classification of Bladder Pain Syndrome (BPS)

Types of BPS were defined based on findings used to document positive signs for the diagnosis of BPS.

The name BPS will be followed by a type indication that consists of two symbols: symbols 1, 2, or 3 indicate findings at cystoscopy with hydrodistention and symbols A, B or C of biopsy findings. 

For example, BPS 3C indicates: –

Score 3 on cystoscopy – Hunner’s lesion on cystoscopy

Score C on biopsy – Biopsy shows inflammatory infiltrates and/or detrusor mastocytosis and/or granulation tissue and/or intrafascicular fibrosis.

Symptom scoring questionnaires for Bladder Pain Syndrome (BPS)

  • University of Wisconsin IC Scale,
  • The O’Leary-Sant IC Symptom Index and
  • IC Problem Index and
  • the Pelvic Pain and Urgency/Frequency Scale.

Pain rating scales: –

  • Numerical rating scores or
  • The McGill pain questionnaire.

Effect of Bladder Pain Syndrome (BPS) on Quality of Life (QoL): -

Patients with BPS can have low self-esteem, sexual dysfunction and reduced QoL.

Patients with BPS may have other coexistent conditions impacting on their QoL.

Conservative measures for Bladder Pain Syndrome (BPS): -

Dietary modification can be beneficial and avoidance of caffeine, alcohol, and acidic foods and drinks should be considered.

Certain foods that worsen pain are alcohol, citrus fruits, coffee, carbonated drinks, tea, chocolate and tomatoes.

If dietary modifications do not help symptoms, these foods can be reintroduced.

Pharmacological treatments for Bladder Pain Syndrome (BPS): -

Oral amitriptyline or cimetidine may be considered when first-line conservative treatments have failed.

Cimetidine is not licensed to treat BPS and should only be commenced by a clinician specialised to treat this condition.

Intravesical treatments for Bladder Pain Syndrome (BPS): -

If conservative and oral treatments have been unsuccessful, other therapies may be added or substituted using an individualised approach.

Options include:

Intravesical lidocaine.

Intravesical hyaluronic acid.

Intravesical injection of botulinum toxin A (Botox).

Intravesical dimethyl sulfoxide (DMSO).

Intravesical heparin.

Intravesical chondroitin sulfate

Lidocaine:-

Lidocaine is a local anaesthetic that acts by blocking sensory nerve fibres in the bladder.

Hyaluronic acid: –

Hyaluronic acid given in a weekly regimen for up to 4 – 10 weeks.

DMSO: –

50% DMSO for two sessions each week for 2 weeks.

Adverse effects include a garlic-like taste and odour on the breath and skin, and bladder spasm.

Full eye examination is needed prior to starting treatment and 6-monthly blood tests for renal, liver and full blood counts are advised.

Further Treatment Options for Bladder Pain Syndrome (BPS): -

Further options should only be considered after referral to a pain clinic and discussion at a multidisciplinary team (MDT) meeting.

Cystoscopic fulguration and laser treatment, and transurethral resection of lesions can be considered if Hunner lesions are identified at cystoscopy.

Neuromodulation treatment for Bladder Pain Syndrome (BPS): -

Neuromodulation (nerve stimulation), in the form of posterior tibial or sacral neuromodulation, may be considered after conservative, oral and/or intravesical treatments have failed, in a multidisciplinary setting.

Posterior tibial nerve stimulation: -

Posterior tibial nerve stimulation (PTNS) is likely to require a fine needle being inserted 5 cm cephalad from the medial malleolus and posterior to the margin of the tibia at the site of the posterior tibial nerve. The treatment regimen is usually weekly for 10–12 weeks.   PTNS is an office-based (outpatient) procedure with no incisions.

Sacral nerve modulation: -

Sacral nerve modulation involves an initial test phase with insertion of a test lead tunnelled under the skin, transmitted onto the nerve roots exiting the S3 foramen, causing stimulation of the pelvic and pudendal nerves.

Both forms of neuromodulation are invasive procedures and are associated with potential risks. 

Other Options: -

Oral cyclosporin A may be considered after conservative, other oral, intravesical and neuromodulation treatments have failed.

Cystoscopy with or without hydrodistension may be considered if conservative and oral treatments have failed.

Cystoscopy is recommended as a treatment rather than solely as a diagnostic tool.

Surgical Options Options for Bladder Pain Syndrome (BPS): -

Major surgery may be considered as last-line treatment in refractory BPS.

Total cystectomy and urinary diversion in the form of supratrigonal cystectomy with bladder augmentation, bowel or supratrigonal cystectomy, and orthotopic neobladder formation will likely need intermittent self-catheterisation.

Patients must be aware of the likelihood of persistent pelvic and pouch pain postsurgery.

Urinary diversion in the form of an ileal conduit (with or without simple cystectomy) will not require intermittent self-catheterisation.

Treatments that are not recommended for Bladder Pain Syndrome (BPS): -

Oral hydroxyzine does not appear to be an effective treatment for BPS.

Oral pentosan polysulfate (PPS) does not appear to be an effective treatment for BPS.

  • PPS is thought to repair the damaged glycosaminoglycan layer, which acts as a protective mechanism for the bladder mucosa.
  • PPS has the adverse effects of diarrhoea, vomiting, rectal bleeding and alopecia.

Long-term antibiotics, intravesical resiniferatoxin, intravesical Bacillus Calmette–Guerin, high-pressure long-duration hydrodistension and long-term oral glucocorticoids are therapies that are not recommended for BPS.

Long-term oral glucocorticoid administration is not recommended due to its long-term adverse effect profile.

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