LpX and LpZ associated hypercholesterolemia in a patient with primary sclerosing cholangitis - A case report.

This case report highlights the complex interplay between cholestatic liver disease and lipoproteins in a 45-year-old male with primary sclerosing cholangitis who presents with severe hypercholesterolemia. Nuclear magnetic resonance lipoprofile revealed marked elevations in lipoprotein X and lipoprotein Z (LpZ), with LpZ being the predominant abnormal lipoprotein. Treatment with evolocumab, a proprotein convertase subtilisin/kexin type 9 (PCSK9) monoclonal antibody, effectively reduced LpZ levels, with a consequent decrease in low-density lipoprotein particle concentration. Subsequent treatment with rosuvastatin 5 mg daily further lowered LpZ levels without exacerbating liver dysfunction. This case emphasizes the importance of distinguishing secondary lipoprotein abnormalities from primary hypercholesterolemia in patients with liver disease, in order to guide personalized therapeutic strategies.