Cholesterol embolization syndrome is a condition in which cholesterol crystals dislodge from atherosclerotic plaques and enter the bloodstream, blocking small to medium arteries in various organs.

Epidemiology

Etiology

• Iatrogenic
  • Vascular interventions, e.g., percutaneous coronary intervention (PCI)
  • Cardiac surgery

Pathophysiology

Atherosclerosis → rupture of atherosclerotic plaque (most commonly from the aorta) → blockage and inflammation of small to medium arteries by cholesterol crystals → formation of multiple small peripheral, muscular, or visceral emboli → end-organ damage

Clinical features

• Features of renal damage
  • Signs of acute kidney injury
• Peripheral skin manifestations
  • Livedo reticularis
  • Necrosis
  • Purpura
  • Blue toe syndrome: ischemia due to small vessel occlusion that manifests as toe discoloration (pulses typically remain palpable as large arteries are unaffected)
• Signs of gastrointestinal involvement (e.g., intestinal ischemia or pancreatitis)
• Signs of CNS involvement (e.g., ischemic stroke or TIA)
• Signs of retinal involvement
  • Hollenhorst plaques on retinal exam
  • Amaurosis fugax

Diagnostics

• CBC with differential
  • Leukocytosis, eosinophilia
  • Anemia
  • Thrombocytopenia
• Renal function tests: ↑ BUN, ↑ creatinine
• Urine studies: proteinuria, hematuria, eosinophiluria
• Biopsy
  • Amorphous, eosinophilic material in the vessel lumen
  • Spindle-shaped spaces (cholesterol clefts)

Treatment