1. Jen Graham 28/03/14

2.  Definitions  Epidemiology  Clinical Assessment  Aetiology  Management

3.  Poorly defined  Initially defined in research setting  Pelvic Pain + Urinary storage symptoms  Heterogenous spectrum of disorders  Inflammation is only present in a small subset of patients  IC vs PBS or BPS

4.  Bladder Pain Syndrome (BPS) is the occurrence of persistent or recurrent pain perceived in the urinary bladder region  accompanied by at least one other symptom e.g. pain worsening with bladder filling, day-time and/or night time urinary frequency  In the absence of “confusable conditions” e.g. urinary tract infection

5.  Mostly women (10:1 F:M)  No difference in race or ethnicity  Genetic component may be present  Age ≥ 18  Median age 42-45 at diagnosis  Associations:  allergies,  functional somatic syndromes - IBS, fibromyalgia, CFS  autoimmune - Sjogren’s syndrome, SLE  depression

6.  Variable due to inconsistent definitions  Difficult to diagnose and treat  No pathological criteria define the disease  Large variation 0.06%-30%  Mainly <1% in most populations

7.  Made on the basis of:  History  Examination  Urinalysis  Cystoscopy with hydrodistension +/- biopsy

9.  Nature of the pain is key  Pain/pressure discomfort perceived to be related to bladder, increasing with increasing bladder content  Located suprapubically but may radiate to groins, vagina, rectum or scrotum  Relieved by voiding, but returns  Aggravated by food or drink  Other LUTS – frequency, urgency, haematuria  Urological diseases (incl. UTI) - Previous pelvic operations - Previous pelvic radiation treatment  Other PMH e.g. Autoimmune disease

10.  Abdomen / bladder  Males:  DRE  Females:  PV for pain mapping of vulval region tenderness of urethra, trigone and bladder superficial/ deep vaginal tenderness tenderness of pelvic floor (levator, adductor)

11.  Validated symptoms score  Can be helpful in monitoring response to treatment

12.  MSU  Urine cytology  EMU x3 (if sterile pyuria)  Chlamydia  Other tests guided by history  e.g. Foreign travel

13.  Under GA  Fill to maximum capacity and distend for 3 minutes at 80-100 cmH2O  Empty and measure volume and look for bleeding  2 nd look only fill to 1-2/3 bladder capacity  Inspect bladder  Cystoscopy + biopsy may differentiate different subtypes  Development of glomerulations is a positive prognostic sign

15.  Inflammation often leading to small capacity fibrotic bladders  Reddened mucosa  Hunner’s Ulcers in 5- 10%  Small vessels radiating to central scar  Scar ruptures at bladder distension leading to waterfall-type bleeding

16.  Likely to be multifactorial 1. Urinary infections used to be thought to be initial insult  UTI/urgency more frequent during childhood in subsequent sufferers 2. Mast cells  ? Causative or secondary  Frequently associated with PBS/IC bladder, also present in non IC bladders  Active allergies exacerbate symptoms 3. Epithelial permeability  Deficiencies in glycosaminoglycan (GAG) layer  Exposes submucosal nerve endings to noxious urine components 4. Neurogenic Inflammation  Abnormal sensory nerve activity 5. Autoimmunity 6. Hormonal

18.  IC / PBS not curable  Try to make patient as self-reliant as possible  Manage expectations of patient  Lots of potential treatments → lots of potential treatment combos  Spontaneous temporary remission can be short lived and unrelated to therapy (up to 20% in placebo studies)

19.  Behavioural  Dietary  Pharmacological  Intravesical  Surgical  Interstitial cystitis database study noted >180 treatment modalities for IC/PBS with poor results in the majority of cases

20.  Behavioural bladder training  in patients who predominately have frequency / urgency with little pain  Diet  no real affect in altering diet (little more than placebo), though there are many lists of foods to avoid  Intravaginal electrical stimulation  effective in alleviating pain  Acupuncture  conflicting evidence, may be beneficial  not a recommended / evidenced based therapy

21.  Analgesics  Often disappointing effect on visceral pain of BPS  Amitriptyline  Blocks h1 histaminergic receptors and decreases mast cell activity.  Decreases painful nociception by inhibition of reuptake of serotonin and noradrenaline  Cimetidine  H2 receptor antagonist  Pentosan polysulphate sodium (Elmiron)  Heparin analogue  Thought to substitute defect in GAG layer

22.  Given via intermittent catheter  Pentosan polysulphate  Glycoprotein replacing deficient GAG layer  Hyaluronic acid (Cystistat)  Chondroitin sulphate  Dimethyl sulphoxide (DMSO)  Chemical solvent that penetrates cell membranes  Claimed to have analgesia, anti-inflammatory and muscle relaxant effects

23.  Hydrodistension  Transurethral resection of Hunner’s ulcers  Intratrigonal botox  Neuromodulation  Denervation procedures  Cystectomy/cystoplasty