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Exclusion criteria for diagnosis of interstitial cystitis, from Campbell's

Connection with other similar urologic diagnoses, from Campbell's

Bladder pain and relation with non-GU pain diagnoses, from Campbell's

Differential and workup to exclude similar diagnoses, from Campbell's

Algorithm for general workup of suspected BPS, from Campbell's

Guideline for thorough evaluation of patient with BPS, from Campbell's

Evaluation

Definitions

• Interstitial cystitis: unpleasant sensation related to urinary bladder associated with LUTS in absence of other causes, more than 6 weeks duration
• Bladder pain syndrome (BPS): suprapubic pain related to bladder filling, associated with other symptoms in the absence of other pathology
• High sensitivity definition: pain, pressure, or discomfort in the pelvic area and daytime urinary frequency 10+ or urgency due to pain, pressure, or discomfort, not fear of wetting (81% sensitive, 54% specific)
• high specificity definition: pain, pressure, or discomfort in the pelvic area and daytime urinary frequency 10+ or urgency due to pain, pressure, or discomfort, not fear of wetting and symptoms did not resolve after treatment with antibiotics and no treatment with hormone injection therapy for endometriosis (48% sensitive, 83% specific)
• Hunner lesion: inflamed area on bladder wall in patients with bladder pain on filling, patients may be divided into those with or without Hunner lesions
• Glomerulations: nonspecific petechial hemorrhages seen with IC/BPS but can be seen in other disorders as well as asymptomatic patients

Related conditions

• Urologic Chronic Pelvic Pain Syndrome: chronic pelvic/genital pain, often accompanied with urinary frequency or urgency, includes prostatitis
• Overactive bladder: urgency with(out) frequency, nocturia, urge incontinence - mainly concerned about urgency, as opposed to bladder pain
• Pelvic Floor Dysfunction: musculoskeletal pain secondary to pelvic floor muscle tightness/spasms

Evaluation/workup

• History: symptom duration, voiding frequency, urgency, location of pain, sexual dysfunction, bowel dysfunction
• Urgency: IC/BPS patients void to avoid pain, OAB patients void to avoid incontinence
• Physical: genital/pelvic exam, locations of masses, tenderness, and hernias
• Urinalysis + urine culture: rule out infection and hematuria
• Baseline symptoms: assess to track response to treatments, can use GUPI or ICSI
• Voiding diary: usually shows low volume urinary frequency, similar to OAB
• Cystoscopy: beneficial to diagnose Hunner lesions
• Urodynamics not required, may be helpful for ruling out confounding diagnoses, may demonstrate hypersensitivity
• THE KEY: IC/BPS is a diagnosis of exclusion, so make sure all other diagnoses (see NIDDK criteria) are excluded

Management (see AUA algorithm)

Behavioral

• Wellness: stress reduction, exercise, warm baths, frequent voiding, and maintaining a "normal" lifestyle
• Physicial therapy: benefits those patients with likely pelvic floor dysfunction, avoid kegels (tightens pelvic floor)
• Dietary triggers: caffeine, alcohol, sweeteners, hot peppers, acidic beverages

Oral medications

• Non-specific pain regimens: pyridium, NSAIDs, acetaminophen, gabapentin, baclofen
• Narcotics: ensure pain contract with single provider, long-acting choices preferable
• Amytriptyline (TCA): 25-100mg QHS, starting with 10mg and titrating up 10mg weekly to minimize side effects (sedation, nausea), avoid if risk of retention, constipation, orthostatic hypotension, or long QT syndrome
• Cimetidine (H2-blocker): 200mg TID, 300mg BID, or 400mg BID, minimal side effects
• Hydroxyzine (H1-blocker): 25mg QHS titrated up to 50mg QHS + 25mg QAM, temporary weakness/sedation noted
• Pentosan polysulfate (Elmiron): 100mg TID, side effects include alopecia, diarrhea, nausea, rash, and pigmentary maculopathy (refer for retinal exam if preexisting ophtho conditions, then periodic exams starting within 6mo treatment)

Intravesical interventions

• Hydrodistension: fill to 80cm for 1-2 minutes, assess for glomerulations (not specific for IC/BPS), be aware of perforation risk, allows for blader inspection and ability to assess bladder capacity (assess for small fibrotic bladder), symptoms may immediately worsen after hydrodistension
• DMSO: 50mL 50% solution intravesical, usually administered as a cocktail weekly for 6 weeks, then monthly for 6 months
• Heparin: mimics the bladder mucopolysaccharide layer, 10mL 10KU intravesical +/- DMSO 3x weekly x3mo
• Lidocaine: can be used with other intravesical therapies, usually not given solo, dosage varies
• Botox: 100U injected intravesically mainly at trigone, discuss need for catheterization, can combine with hydrodistension

Hunner lesion-specific therapies

• Cyclosporine: 2.5-5mg/kg daily for 3-6 months then 1.5-3mg/kg daily maintenance dose, side effects include renal dysfunction, hypertension, immunosuppression, gum hyperplasia, alopecia, tremor, gout, aphthous ulcers, cutaneous lymphoma
• Intravesical therapies: fulguration (laser/bugbee) or triamcinolone injection up to 60mg (10-40mg/mL) injected as 0.5mL amounts around lesion

Surgical interventions

• Sacral neurostimulation: appropriate in correctly selected patient, perform trial run first
• PTNS: can consider but minimal evidence showing benefit
• Urinary diversion: consider only when more conservative therapies have failed, heavily weigh risks vs benefits, symptoms may improve with diversion without cystectomy, (distension and/or urinary exposure may be the cause of symptoms), patients with systemic pain syndromes less likely to respond to surgical management
• Other options: properly selected patients may benefit from augment or supratrigonal cystectomy

Treatments to NOT recommend

• Antibiotics: no benefit, severe longterm risks
• BCG: no benefit, may worsen voiding symptoms
• Hydrodistension: do not offer for high pressure (> 80-100cm) or long duration (> 10min), increases risk for rupture and sepsis
• Steroids: risks outweigh benefits