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atheroembolism; cholesterol embolism; aortic atheroembolism

Etiology: - occurs in patients with severe atherosclerosis a) post arterial procedure - angiogram - arterial catheterization (coronary angiography) - cardiac surgery - aortic surgery b) spontaneously, i.e. large abdominal aortic aneurysm c) may also occur several weeks after starting warfarin therapy Epidemiology: - most patients are > 60 years of age Pathology: 1) embolization involves lodging of cholesterol crystals in small arterioles & capillary beds 2) skin biopsy may show needle-shaped cholesterol crystals within dermal arteries (histology image) [6] 3) inflammatory infiltrate 4) intimal thickening 5) perivascular fibrosis 6) lipid-laden giant cells 7) embolization occurs commonly to: a) kidney b) spleen c) pancreas 8) embolization occurs less commonly to: a) central nervous system - retinal artery - Hollenhorst plaque b) muscles Clinical manifestations: 1) acute renal failure (ARF) a) after 24 hr (slower onset than ARF secondary to radiographic contrast media) b) severe hypertension may occur due to atheroembolism to the kidney c) poor prognosis for recovery of renal function 2) livedo reticularis over the legs & thighs is common [2] 3) purple toes (blue toe syndrome), digital gangrene - petechiae on plantar surface of feet (image) [6] 4) visual changes, retinal changes, Hallenhorst plaque (pathognomonic) [2] 5) cerebrovascular accident 6) GI bleed 7) abdominal pain, pancreatitis 8) fatigue 9) generalized myalgias 10) low-grade fever may occur Laboratory: 1) complete blood count (CBC) - transient eosinophilia may be present - leukocytosis may be noted 2) renal function tests - increased serum creatinine 3) urinalysis: - eosinophiluria may be present - other findings may include proteinuria, hematuria, leukocytes in urine, or granular casts, leukocyte casts, or erythrocyte casts [2] 4) CH50 may be abnormal due to complement activation by cholesterol crystals - complement C3 in serum may be low - complement C4 in serum may be low 5) erythrocyte sedimentation rate may be elevated Special laboratory: - biopsy of infarcted skin, nerve, kidney, or muscle Differential diagnosis: - contrast nephropathy: no hypocomplementemia - acute tubular necrosis: no hypocomplementemia - presence of renal tubular epithelial cells in urine supports diagnosis of acute tubular necrosis - cholesterol emboli in the kidney may result in hypertension Management: 1) no specific therapy 2) aggressive control of cardiovascular risk factors a) control of hypertension b) correction of dyslipidemia c) glycemic control in diabetics d) smoking cessation [2] 3) pharmaceutical agents a) acute (limited success) - vasodilators - antiplatelet agents (iloprost) - low molecular weight dextran - glucocorticoids [2] b) long-term - antiplatelet therapy (aspirin vs clopidogrel) - statin 4) LDL apheresis is investigational [2] 5) surgical prophylaxis of further episodes a) repair of aneurysm b) removal of an ulcerated plaque 6) digital necrosis & gangrene require amputation

Related

blue toe syndrome cholesterol livedo reticularis; livedoid vasculopathy; mottled skin; cutis marmorata

Specific

atheroembolic renal failure Hollenhorst plaque

General

atherosclerosis peripheral arterial disease; peripheral artery atherosclerosis (PAD) embolism

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 535
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2021 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  3. Kronzon I, Saric M. Cholesterol embolization syndrome. Circulation. 2010 Aug 10;122(6):631-41 PMID: 20697039
  4. Saric M, Kronzon I. Cholesterol embolization syndrome. Curr Opin Cardiol. 2011 Nov;26(6):472-9. Review. PMID: 21993354
  5. Quinones A, Saric M. The cholesterol emboli syndrome in atherosclerosis. Curr Atheroscler Rep. 2013 Apr;15(4):315. Review. PMID: 23423524
  6. Rashid RM, Barros BS (images) Hidden Heart Disease: 19 Dermatologic Clues You Should Know. Medscape. June 13, 2017. http://reference.medscape.com/slideshow/hidden-heart-disease-6004452
  7. Stefan G, Zugravu A, Stancu S, et al. Atheroembolic kidney disease: the under-recognized silent killer. Clin Case Rep. 2021;9:1824-25. PMID: 33768956