Before treatment, measure ALT, AST, alkaline phosphatase, and total bilirubin; and then ALT and AST regularly as recommended.

For ALT or AST > 3x and <5x ULN:

• Confirm elevation with a repeat measurement within one week. If confirmed, reduce the dose and obtain additional liver-related tests if not already measured (such as alkaline phosphatase, total bilirubin, and INR). Repeat tests weekly and withhold dosing if there are signs of abnormal liver function (increase in bilirubin or INR), if transaminase levels rise above 5x ULN, or if transaminase levels do not fall below 3x ULN within approximately 4 weeks. In these cases of persistent or worsening abnormalities, also investigate to identify the probable cause.  
• If resuming JUXTAPID after transaminases resolve to <3x ULN, consider reducing the dose and monitor liver-related tests more frequently. 

For ALT or AST >5x ULN: 

• Withhold dosing, obtain additional liver-related tests if not already measured (such as alkaline phosphatase, total bilirubin, and INR), and investigate to identify the probable cause.  
• If resuming JUXTAPID after transaminases resolve to <3x ULN, reduce the dose and monitor liver-related tests more frequently. 

If transaminase elevations are accompanied by clinical symptoms of liver injury (such as nausea, vomiting, abdominal pain, fever, jaundice, lethargy, flu-like symptoms), increases in bilirubin ≥2x ULN, or active liver disease, discontinue treatment with JUXTAPID and investigate to identify the probable cause. 

Patients with end-stage renal disease receiving dialysis should not exceed 40 mg daily since lomitapide exposure in these patients increased approximately 50% compared with healthy volunteers. Effects of mild, moderate, and severe renal impairment, including those with endstage renal disease not yet receiving dialysis, on lomitapide exposure have not been studied. However, it is possible that patients with renal impairment who are not yet receiving dialysis may experience increases in lomitapide exposure exceeding 50%. 

Initiate treatment at 5 mg once daily. Titrate dose based on acceptable safety/tolerability: increase to 10 mg daily after at least 2 weeks; and then, at a minimum of 4-week intervals, to 20 mg, 40 mg, and up to the maximum recommended dose of 60 mg daily. 

To reduce the risk of developing a fat-soluble nutrient deficiency due to JUXTAPID’s mechanism of action in the small intestine, patients treated with JUXTAPID should take daily supplements that contain 400 international unit’s vitamin E and at least 200 mg linoleic acid, 210 mg alpha-linolenic acid (ALA), 110 mg eicosapentaenoic acid (EPA), and 80 mg docosahexaenoic acid (DHA).