What are the symptoms and treatment options for end-stage cystitis?

End-Stage Cystitis: Symptoms and Management

The term "end-stage cystitis" most accurately refers to severe, refractory interstitial cystitis/bladder pain syndrome (IC/BPS) that has progressed to bladder contracture with severely reduced functional capacity, representing the most advanced manifestation of chronic bladder inflammation requiring consideration of major surgical intervention 1.

Clinical Presentation of End-Stage Disease

Bladder contracture represents the hallmark of end-stage cystitis, characterized by:

• Severe bladder capacity reduction under anesthesia, often with a small fibrotic bladder that has lost elasticity 1
• Intractable pelvic pain that is constant and unrelenting, perceived in the suprapubic region, bladder, urethra, vulva/vagina (in women), perineum, and lower abdomen 1
• Extreme urinary frequency with severely diminished voided volumes, often voiding every 15-30 minutes during waking hours 1
• Severe nocturia disrupting sleep multiple times per night 1
• Pain that worsens with bladder filling and may only partially improve with voiding, unlike earlier stages where voiding provides relief 1

Additional Severe Symptoms

• Hunner's lesions may be present on cystoscopy, appearing as reddened, inflamed areas with small vessels radiating toward a central scar, often with fibrin deposits 1
• Persistent hematuria that may occur with minimal bladder distension 1
• Complete loss of quality of life with inability to work, maintain relationships, or perform daily activities 1
• Severe psychological distress including depression and anxiety related to chronic, uncontrolled pain 1

Treatment Approach for End-Stage Disease

When Conservative Measures Have Failed

Major surgery should be considered only after all other therapeutic options have been exhausted and symptoms are definitively bladder-centric 1. The best-documented predictors of surgical success are presence of Hunner lesions and small bladder capacity under anesthesia 1.

Surgical Options

Urinary diversion with or without cystectomy may be undertaken in carefully selected patients 1. Key considerations include:

• Patient selection is paramount to surgical outcome—patients must have bladder-centric symptoms rather than generalized pelvic pain 1
• Urinary diversion relieves frequency and nocturia, and sometimes can relieve pain, though outcomes are variable 1
• Risks include persistent pain that can lead to devastating outcomes including suicide, surgical complications, or new symptoms with lifelong significant bother 1
• Substitution cystoplasty (bladder augmentation) is an alternative to complete diversion 1

Pre-Surgical Considerations

Before proceeding to major surgery, ensure the following have been attempted:

• Bladder hydrodistension with fulguration or triamcinolone injection of Hunner's lesions if present 1
• Intravesical DMSO (the only FDA-approved intravesical therapy), instilled as 50 mL every 2 weeks until maximum relief, then at increased intervals 2
• Oral pentosan polysulfate sodium (the only FDA-approved oral therapy for IC/BPS), though adverse events include alopecia, diarrhea, and potential retinal changes 3, 4
• Neuromodulation (sacral or pudendal nerve stimulation) for frequency/urgency symptoms, though less effective for pain 1
• Cyclosporine A or intradetrusor botulinum toxin A as fifth-line options, though patients must accept possible need for intermittent self-catheterization with botulinum toxin 1

Critical Management Principles

Bladder contracture specifically requires 1:

• Postponement of any intravesical instillations
• Lidocaine for sedation and analgesia
• Bladder enlargement procedures if conservative management fails

Common Pitfalls to Avoid

• Do not offer long-term oral antibiotics in culture-negative patients, as this provides no benefit and risks antibiotic resistance 1
• Do not offer intravesical BCG outside investigational settings due to lack of efficacy and risk of serious adverse events including sepsis 1
• Do not offer long-term systemic glucocorticoids as primary treatment due to serious adverse events (diabetes, pneumonia, hypertension) 1
• Avoid surgery in patients with generalized pelvic pain rather than bladder-specific symptoms, as outcomes are poor 1

The progression to end-stage disease with bladder contracture represents a devastating outcome that profoundly impacts mortality risk through suicide and quality of life through unrelenting pain and urinary symptoms 1. Aggressive multimodal therapy earlier in the disease course may prevent progression to this irreversible stage.