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Cleveland Clinic Journal of MedicineXanthomas: Differentiating Atherogenic from Nonatherogenic

Lipid Dysregulation in Primary Biliary Cholangitis: Implications Beyond Liver Disease

In a detailed examination of a patient with primary biliary cholangitis (PBC), an autoimmune liver disease, various cutaneous manifestations were analyzed to decipher the underlying lipid abnormalities. This case study highlights the diverse presentation of xanthomas, which serve as clinical indicators of different types of dyslipidemia. Understanding these markers is crucial in directing the appropriate management of lipid levels, particularly in conditions like PBC where lipid profiles deviate from typical dyslipidemic patterns without increasing cardiovascular risks.

Key Points:

  • A 42-year-old woman with primary biliary cholangitis presented with multiple types of xanthomas, which were crucial in diagnosing her lipid abnormalities.
  • The patient exhibited plane xanthomas around the eyes, xanthoma striatum palmare on the palms, cobblestone-like nodules on hand joints, and eruptive xanthomas on the buttock—each indicating different lipid disorders.
  • Lab results revealed markedly elevated liver enzymes, bilirubin levels, and extremely high cholesterol and LDL levels, with normal renal function and protein levels.
  • Xanthomas are caused by oxidized lipid deposits in the dermis and their types provide clues about underlying dyslipidemia, which can be atherogenic or nonatherogenic.
  • Lipid abnormalities in primary biliary cholangitis are often due to lipoprotein X, an abnormal LDL particle that does not increase cardiovascular risk, distinguishing it from other lipid disorders.
  • The treatment for the patient’s pruritus associated with PBC is cholestyramine, whereas liver transplant remains the definitive treatment for PBC itself.
  • Statin therapy, typically used for managing hypercholesterolemia, is not advised in PBC unless benefits clearly outweigh the risks, due to the unique lipid profile in these patients.

“The lipid accumulation seen in primary biliary cholangitis is due to a unique lipid subfraction known as lipoprotein X, an abnormal nonatherogenic LDL particle that is not associated with an increased risk of cardiovascular disease.”

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