What is the recommended treatment for a urinary tract infection in a patient with Ehlers-Danlos syndrome?

Treatment of Urinary Tract Infections in Patients with Ehlers-Danlos Syndrome

For patients with Ehlers-Danlos syndrome (EDS) who develop a urinary tract infection, the recommended first-line treatment is nitrofurantoin monohydrate/macrocrystals 100 mg twice daily for 5 days, as it has minimal resistance patterns and limited collateral damage to gut flora. 1

Understanding UTIs in Ehlers-Danlos Syndrome

Patients with EDS are particularly prone to urinary tract complications due to connective tissue abnormalities that can lead to:

• Bladder diverticula (common in EDS) 2, 3
• Vesicoureteral reflux 2
• Pelvic organ prolapse (prevalence 29-75% in EDS) 2
• Urinary retention 4
• Recurrent UTIs 5, 3

These anatomical abnormalities can complicate UTI treatment and increase recurrence risk, making proper antibiotic selection crucial.

First-Line Treatment Options

1. Nitrofurantoin monohydrate/macrocrystals

   • Dosage: 100 mg twice daily
   • Duration: 5 days
   • Advantages: Minimal resistance patterns, limited impact on gut flora
   • Contraindications: Avoid if GFR <30 ml/min, suspected pyelonephritis, or in infants <4 months 1

2. Trimethoprim-sulfamethoxazole (TMP-SMX)

   • Dosage: 160/800 mg (one double-strength tablet) twice daily
   • Duration: 3 days for uncomplicated cystitis; 10-14 days for complicated UTIs 1, 6
   • Only use if local resistance is <20% and not used for UTI treatment in previous 3 months 1
   • For adults with impaired renal function, dosage should be reduced according to creatinine clearance 6

3. Fosfomycin trometamol

   • Dosage: 3 g as a single dose
   • Note: Somewhat lower efficacy than other options; avoid if pyelonephritis is suspected 1

Special Considerations for EDS Patients

• Extended treatment duration: Consider 10-14 days of therapy for EDS patients due to anatomical abnormalities that may complicate infection clearance 6

• Careful monitoring: Due to the high prevalence of bladder diverticula in EDS patients, which can harbor bacteria and lead to persistent or recurrent infections 3

• Fluoroquinolones (e.g., ciprofloxacin): Should be reserved as alternative options due to concerns about promoting resistance 1, but may be necessary for complicated UTIs in EDS patients with anatomical abnormalities

Management Algorithm

1. Obtain urine culture before starting antibiotics

   • Essential for EDS patients due to higher risk of complicated UTIs and recurrence
   • Allows for targeted therapy based on susceptibility

2. Initial empiric therapy while awaiting culture results:

   • Nitrofurantoin 100 mg twice daily (if no contraindications)
   • If pyelonephritis suspected or GFR <30 ml/min: TMP-SMX or fluoroquinolone

3. Adjust therapy based on culture results:

   • Continue initial therapy if organism is susceptible
   • Switch to appropriate antibiotic based on susceptibility if resistant

4. Duration of therapy:

   • Uncomplicated cystitis: 5 days
   • Complicated UTI (likely in many EDS patients): 10-14 days 6

5. Follow-up urine culture after completion of therapy to ensure clearance of infection

Prevention Strategies

• Increased fluid intake (additional 1.5L daily) 1
• Consider prophylactic antibiotics for recurrent UTIs:
  • Nitrofurantoin 50-100 mg daily
  • TMP-SMX 40/200 mg daily
  • Post-coital single dose when UTIs are related to sexual activity 1

Caution

EDS patients with bladder diverticula may experience treatment failures with standard regimens due to bacterial persistence in diverticula. In cases of recurrent UTIs despite appropriate therapy, urologic evaluation for anatomic abnormalities is warranted, as surgical intervention may sometimes be necessary in severe cases 3.