Venxxiva (tiopronin) — Highmark
severe homozygous cystinuria
Preferred products
- generic tiopronin
Initial criteria
- Member weighs 20 kg or more
- Diagnosis of cystinuria (ICD-10: E72.01) classified as severe homozygous, supported by at least one 24-hour urine collection with urinary cystine excretion > 400 mg/day
- Experienced therapeutic failure to all of the following regimens: increased fluid intake of at least 4 liters per day AND dietary modifications (e.g., reduced sodium and animal protein intake) AND urine alkalinization (e.g., with potassium citrate) to achieve urinary pH of 7.0
- If the request is for Venxxiva, member has experienced therapeutic failure or intolerance to plan‑preferred generic tiopronin
Reauthorization criteria
- Prescriber attests that member’s urine cystine concentration has decreased from baseline OR member’s production of cystine stones has decreased from baseline
Approval duration
initial: up to 6 months (Delaware Commercial fully-insured and ACA members: 12 months); reauthorization: up to 12 months