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TEXTBOOK PRESENTATION

A classic presentation is a white man over age 60 with hypertension, smoking, and vascular disease in whom livedo reticularis and acute or subacute kidney injury develop after an inciting event.

DISEASE HIGHLIGHTS

  1. Secondary to cholesterol crystal embolism from an atherosclerotic aorta

  2. 3 syndromes: abrupt onset of kidney injury after an inciting event (such as angiography), subacute worsening of kidney function a few weeks after an event, and chronic kidney impairment

  3. Risk factors include

    1. Male sex

    2. Age > 60 years

    3. Hypertension

    4. Smoking

    5. Diabetes mellitus

    6. Vascular disease

  4. Can occur spontaneously or after vascular surgery procedures, angiograms (especially coronary angiograms), and with anticoagulation

  5. Incidence probably quite low (< 1–2%) but may be as high as 5–6% in high-risk patients.

  6. Clinical manifestations (from 5 case series)

    1. Skin lesions (livedo reticularis) in 35–90%

    2. Gastrointestinal symptoms in 8–30%

    3. Eosinophilia in 22–73%

    4. CNS involvement in 4–23%

    5. Dialysis needed in 28–61%

EVIDENCE-BASED DIAGNOSIS

  1. Kidney or skin biopsy

  2. Can sometimes be diagnosed on fundoscopic exam

TREATMENT

  1. Best approach beyond supportive therapy unknown

  2. Avoid anticoagulation

  3. Consider aggressive lipid management

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