Intersitial cystitis - An unpleasant sensation (pain, pressure, discomfort) perceived to be related to the urinary bladder, associated with lower urinary tract symptoms of more than six weeks duration, in the absence of infection or other identifiable causes.

• AUA guidelines based on above SUFU definition of IC
• NIDDK diagnostic criteria requires cystoscopic findings; used for research, but too restrictive for clinical use
• Rule out infections, other conditions like malignancy
• Pathophysiology - leaky epithelium/defect in glycosaminoglycan layer with neurogenic inflammation from neuropeptides like substance P?, depolarization from urinary potassium, increased sympathetic activity in bladder?
• Pelvic organ cross-sensitization (e.g. cross-talk with IBS, bowel disorders)

History 

• Symptoms
• Voiding symptoms
• Classic - Pain on bladder filling; voiding due to discomfort
• Pain levels
• O'Leary-Sant symptom scale
• Previous pelvic surgery 
• Previous urinary tract infection
• Bladder history and urologic diseases
• Location of pelvic pain and relationship to bladder filling and emptying 
• Characteristics, onset, correlation of pain with other events 
• Previous pelvic irradiation 
• Autoimmune diseases 
• Associated syndromes (irritable bowel, fibromyalgia, chronic fatigue) 

Physical Examination 

• Standing: kyphosis, scars, hernia 
• Supine: abduction and adduction of hips, hyperesthetic areas 
• Females: vaginal examination with pain mapping of vulvar region, vaginal palpation for tenderness of the bladder, urethra, levator and adductor muscles of the pelvic floor 
• Males: digital rectal examination with pain mapping of the scrotal-anal region and palpation of tenderness of the bladder, prostate, levator and adductor muscles of the pelvic floor and scrotal contents 

Labs: UA/culture, cytology if risk of malignancy (e.g. smoking and > 40)

Cystoscopy +/- hydrodistention, and UDS to aid complex diagnosis - but not sensitive/specific for IC diagnosis

• Cystoscopy can identify Hunner lesion (weakness that can ulcerate on bladder distention)
• Glomerulations not specific/sensitive for IC (seen after radiation, cancer, chemical exposure)

Diagnostic markers - unclear! ?mast cells, NO, urine APF, intravesical KCl solution (pain may indicate epithelial abnormality)

First-line - Self-care, behavioral modifications, stress management

• Diet (mixed evidence for efficacy) - avoid alcohol, caffeine, spicy foods, acidic foods (eg tomato) - bladder irritants
• Tums to alkalinize urine
• Prelief to prevent bladder pain from foods
• Quercitin supplement

Second-line - physical therapy (muscular trigger points, contractures, scars, etc.). Avoid Kegel exercises. Initiate pain management (multi-modal).

• PO medications: amitriptyline, cimetidine, hydroxyzine, pentosan polysulfate (Elmiron)
• Intravesical: DMSO, heparin, lidocaine

Third-line - cystoscopy with short duration low pressure hydrodistention (increases bladder blood flow, will see tiny petechiae form afterwards). Fulgurate Hunner's lesions if present

• 50% of patients note improvement in symptoms with hydrodistention

Fourth-line - Botox (risk of self-cath), neurostimulation

Fifth-line - PO cyclosporine A

Sixth-line - major surgery (e.g. urinary diversion/cystectomy)