• Inborn errors of metabolism

Zokinvy Generic Name & Formulations

General Description

Lonafarnib 50mg, 75mg; caps.

Pharmacological Class

Farnesyltransferase inhibitor.

How Supplied


Generic Availability


Zokinvy Indications


To reduce the risk of mortality in Hutchinson-Gilford progeria syndrome. Treatment of processing-deficient progeroid laminopathies with either: heterozygous LMNA mutation with progerin-like protein accumulation or homozygous or compound heterozygous ZMPSTE24 mutations.

Limitations of Use

Not for other progeroid syndromes or processing-proficient progeroid laminopathies.

Zokinvy Dosage and Administration

Adults and Children

<12months (or BSA <0.39m2): not established. Swallow whole with water; if unable to swallow, mix contents with Ora Blend SF®, Ora-Plus®, orange juice or applesauce. ≥12 months (with BSA ≥0.39m2): initially 115mg/m2 twice daily with morning and evening meals; increase to 150mg/m2 twice daily after 4 months; see full labeling. Round all total daily dosages to the nearest 25mg increment. Concomitant weak CYP3A inhibitors (if unavoidable): reduce to or continue Zokinvy at initial dose of 115mg/m2 twice daily. Other dose modifications: see full labeling.

Zokinvy Contraindications


Concomitant strong or moderate CYP3A inhibitors or inducers. Concomitant midazolam, lovastatin, simvastatin, or atorvastatin.

Zokinvy Boxed Warnings

Not Applicable

Zokinvy Warnings/Precautions


Periodically monitor electrolytes, CBCs, liver enzymes, and renal function during therapy. Perform ophthalmological evaluation regularly and at onset of new visual changes during therapy. Renal or hepatic impairment. Embryo-fetal toxicity. Advise females of reproductive potential to use effective contraception during therapy. Pregnancy: avoid. Nursing mothers.

Zokinvy Pharmacokinetics

See Literature

Zokinvy Interactions


See Contraindications. Temporarily discontinue Zokinvy 10–14 days before and 2 days after administration of midazolam. Avoid concomitant weak CYP3A inhibitors (see Adults and Children), CYP2C9 inhibitors; if unavoidable, monitor closely for arrhythmias, syncope, heart palpitations. Avoid grapefruit or Seville oranges. Avoid concomitant sensitive CYP3A or CYP2C19 substrates; if unavoidable, monitor and reduce dose of substrate. Potentiates loperamide; do not exceed loperamide 1mg once daily when first coadministered; increase cautiously. Potentiates P-gp substrates (eg, digoxin, dabigatran); monitor and reduce dose of substrate.

Zokinvy Adverse Reactions

Adverse Reactions

Vomiting, diarrhea, infection, nausea, decreased appetite, fatigue, upper respiratory tract infection, abdominal pain, musculoskeletal pain, electrolyte abnormalities, decreased weight, headache, myelosuppression, increased AST/ALT, decreased blood bicarbonate, cough, hypertension; nephrotoxicity, retinal toxicity, impaired fertility.

Zokinvy Clinical Trials

See Literature

Zokinvy Note

Not Applicable

Zokinvy Patient Counseling

See Literature