Generic Name and Formulations:
Lomitapide mesylate 5mg, 10mg, 20mg; caps.

Company:
Aegerion Pharmaceuticals
Indications for JUXTAPID:
As an adjunct to low-fat diet and other lipid-lowering treatments, including LDL apheresis where available, to reduce LDL-C, total cholesterol, apo B, and non-HDL-C in patients with homozygous familial hypercholesterolemia (HoFH). Limitations of use: not for patients with hypercholesterolemia who do not have HoFH. Effect on cardiovascular morbidity, mortality has not been determined.

Adult Dose for JUXTAPID:
Before starting: measure ALT, AST, alkaline phosphatase, total bilirubin; obtain (–) pregnancy test; initiate low-fat diet supplying <20% of energy from fat. Swallow whole, take 2hrs after PM meal. Initially 5mg once daily. Titrate dose based on safety and tolerability: increase to 10mg daily after at least 2 weeks; and then at a minimum of 4-week intervals, to 20mg, 40mg, and up to max 60mg daily. Supplement with daily Vit.E, linoleic acid, ALA, EPA, and DHA. ESRD on dialysis or baseline mild hepatic impairment: max 40mg daily. Concomitant weak CYP3A4 inhibitors (eg, alprazolam, amiodarone, amlodipine, atorvastatin, bicalutamide, cilostazol, cimetidine, cyclosporine, fluoxetine, fluvoxamine, ginkgo, goldenseal, isoniazid, lapatinib, nilotinib, oral contraceptives, pazopanib, ranitidine, ranolazine, tipranavir/ritonavir, ticagrelor, zileuton): max 30mg daily. Dose adjustments for elevated transaminases: see full labeling.

Children's Dose for JUXTAPID:
<18yrs: not established.

Pharmacological Class:
Microsomal triglyceride transfer protein (MTP) inhibitor.

Rems:
YES

Contraindications:
Pregnancy (Cat.X). Concomitant moderate (eg, amprenavir, aprepitant, atazanavir, ciprofloxacin, crizotinib, darunavir/ritonavir, diltiazem, erythromycin, fluconazole, fosamprenavir, imatinib, verapamil) or strong (eg, boceprevir, clarithromycin, conivaptan, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, voriconazole) CYP3A4 inhibitors. Moderate to severe hepatic impairment, active liver disease, unexplained persistent elevations of serum transaminases.

Warnings/Precautions:
Elevations in transaminases and increases in hepatic fat may occur; measure ALT, AST, alkaline phosphatase, total bilirubin before initiating therapy and then ALT, AST regularly and before any dose increase. Adjust dose if ALT or AST are ≥3xULN; discontinue if clinically significant liver toxicity. Adhere to low-fat diet to reduce risk of GI effects. Galactose intolerance, Lapp lactase deficiency, glucose-galactose malabsorption: avoid. Chronic bowel or pancreatic diseases that predispose to malabsorption. Renal impairment. Females: use effective contraception. Nursing mothers: do not use.

Interactions:
See Contraindications and Adults. Potentiated by CYP3A4 inhibitors (omit grapefruit juice). Reduce dose of simvastatin by 50%, consider reducing lovastatin dose. Potentiates warfarin; monitor INR. Consider dose reduction of P-gp substrates. Max one alcoholic drink/day. Caution with other hepatotoxic drugs. Separate bile acid sequestrants by at least 4hrs.

Adverse Reactions:
GI upset, dyspepsia, abdominal pain; hepatotoxicity (including steatohepatitis, hepatic failure) possible, reduced absorption of fat soluble vitamins.

Note:
Juxtapid REMS Program: only certified providers and pharmacies may prescribe and distribute.

Metabolism:
Hepatic (CYP3A4; 1A2, 2B6, 2C8, 2C19 (minor); 99.8% protein bound.

Elimination:
Renal, fecal.

Generic Availability:
NO

How Supplied:
Caps—28