Lomitapide (Juxtapid) is a microsomal triglyceride transfer protein (MTP) inhibitor, used for the treatment of homozygous familial hypercholesterolemia (HoFH).
How to Use Lomitapide (Juxtapid)
Standard Dosing Regimen
Lomitapide is an oral capsule formulation. The recommended dosage is once daily, and it should be taken on an empty stomach at least 2 hours before dinner. The initial dose is 5mg, and it can be gradually adjusted to a maximum dose of 60mg based on tolerability.
Important Precautions
It must be used in combination with a low-fat diet (fat provides ≤20% of total calories).
During treatment, regular monitoring of liver function, fat absorption status, and vitamin E levels is required.
Hospitalization for monitoring is necessary in the early stage of treatment to assess adverse reactions.
Dose Escalation Regimen
Lomitapide requires slow titration to reduce gastrointestinal adverse reactions.
Weeks 1-2: 5mg once daily.
Weeks 3-4: 10mg once daily.
Weeks 5-6: 20mg once daily.
Week 7 and beyond: It can be increased to 40mg based on tolerability, with a maximum of no more than 60mg once daily.
Administration Timing
Take it at a fixed time, preferably at least 2 hours before dinner.
If a dose is missed, do not make up for it; take the next dose at the originally scheduled time.
If vomiting occurs after taking the medicine, do not make up for the missed dose.
Dose Adjustment of Lomitapide (Juxtapid)
Hepatic Dysfunction
ALT or AST > 3×ULN but ≤ 5×ULN: Maintain the current dose and increase the monitoring frequency.
ALT or AST > 5×ULN but ≤ 8×ULN: Reduce the dose by half or suspend administration.
ALT or AST > 8×ULN: Discontinue the medication permanently.
Fat Malabsorption
If severe steatorrhea or malnutrition symptoms occur: Consider reducing the dose or suspending administration.
Supplement fat-soluble vitamins (A, D, E, K) and essential fatty acids.
Concomitant Use with CYP3A4 Inhibitors
Strong CYP3A4 inhibitors (e.g., ketoconazole): The maximum dose should not exceed 30mg.
Moderate CYP3A4 inhibitors (e.g., erythromycin): The maximum dose should not exceed 40mg.
Liver Transplant Patients
The initial dose should not exceed 5mg.
The maximum dose should not exceed 40mg.
More frequent monitoring of liver function is required.
Use in Special Populations for Lomitapide (Juxtapid)
Pregnancy Risk
It is contraindicated during pregnancy, as it may cause fetal malformations.
Women of childbearing age must use effective contraceptive measures during treatment and within 2 weeks after discontinuing the medication.
If accidental pregnancy occurs, discontinue the medication immediately and seek medical attention.
Use During Lactation
It is not yet known whether lomitapide is excreted into human milk.
It is recommended to avoid breastfeeding during treatment and within 2 weeks after discontinuing the medication.
Patients with Renal Impairment
Mild to moderate renal impairment: No dose adjustment is required.
Severe renal impairment (eGFR < 30mL/min): Use with caution and monitor closely.
