What is the treatment for Lymphoplasmacytic (inflammatory) interstitial cystitis?

Treatment for Lymphoplasmacytic Interstitial Cystitis

Amitriptyline is the first-line pharmacologic treatment for lymphoplasmacytic interstitial cystitis, starting at 10mg and titrating gradually to 75-100mg as tolerated. 1

Pharmacologic Treatment Options

First-Line Medications

• Amitriptyline: Start at low doses (10mg) and titrate gradually to 75-100mg as tolerated
  • Mechanism: Provides pain relief through multiple pathways
  • Side effects: Sedation, drowsiness, and nausea 1

Second-Line Medications

• Pentosan polysulfate sodium (Elmiron):
  • FDA-approved specifically for IC/BPS
  • Dosage: 100mg three times daily
  • Clinical efficacy: 38% of patients had >50% improvement in bladder pain vs 18% with placebo
  • Must be taken continuously for relief 1, 2

Additional Pharmacologic Options

• Anticholinergics (darifenacin, fesoterodine, solifenacin, tolterodine, or trospium) for overactive bladder symptoms 1
• NSAIDs for pain relief 1
• Cimetidine, hydroxyzine, and cyclosporine A as adjunctive therapies 1

Non-Pharmacologic Therapies

Behavioral Modifications

• Stress management practices
• Bladder training and urge suppression techniques
• Dietary modifications to identify and avoid trigger foods 1

Physical Therapy

• Manual physical therapy techniques for pelvic floor tenderness (Grade A evidence) 1

Fluid Management

• Modify concentration/volume of urine through fluid restriction or hydration
• Reduce caffeine intake to decrease voiding frequency 1

Intravesical Therapies

FDA-Approved Options

• Dimethyl Sulfoxide (DMSO):
  • Administration: Bladder instillation
  • Frequency: Every two weeks until maximum relief is obtained
  • Properties: Anti-inflammatory and analgesic 1

Other Intravesical Options

• Heparin
• Lidocaine
• Glycosaminoglycan substitution treatments 1, 3

Procedural Interventions

For Patients with Hunner Lesions

• Cystoscopy with fulguration of Hunner lesions
• Hydrodistention of the bladder 1

For Severe Refractory Cases

• Consider surgical options only after conservative treatments have failed for at least 3 years
• If bladder capacity under general anesthesia is >400 ml and pain is the major symptom, denervation by cystolysis may be considered
• If bladder capacity under general anesthesia is <400 ml, supratrigonal cystectomy and substitution colocystoplasty may be considered 4

Treatment Monitoring and Adjustment

Assessment Schedule

• Evaluate treatment efficacy every 4-12 weeks using validated symptom scores
• Discontinue ineffective treatments and adjust therapy based on symptom response and side effects 1

Diagnostic Follow-up

• Regular upper tract imaging (periodic ultrasound) to monitor for complications
• Consider urine culture if pain persists beyond 2 weeks
• Consider cystoscopy if hematuria is present, Hunner lesions are suspected, or symptoms are refractory to initial treatment 1

Treatment Algorithm

1. Initial Approach: Start amitriptyline at 10mg and implement behavioral modifications, dietary changes, and stress management
2. After 4-6 weeks: If inadequate response, titrate amitriptyline up to 75-100mg as tolerated
3. After 12 weeks: If still inadequate response, add pentosan polysulfate sodium 100mg three times daily
4. Concurrent therapy: Consider physical therapy for pelvic floor dysfunction
5. For persistent symptoms: Add intravesical therapies, starting with DMSO
6. For patients with Hunner lesions: Consider cystoscopy with fulguration and/or hydrodistention
7. For refractory cases: Consider immunomodulators like cyclosporine or surgical options as last resort

Important Caveats

• Rule out infection with urinalysis and urine culture before initiating treatment
• Flank pain is not typical of IC/BPS and warrants thorough evaluation of the upper urinary tract
• Pentosan polysulfate sodium may cause maculopathy with long-term use, related to cumulative exposure
• Multimodal approaches combining pharmacologic and non-pharmacologic therapies are most effective
• Surgical interventions should be reserved for severe cases unresponsive to conservative management for at least 3 years