Clinical features and diagnosis

This condition predominantly affects the elderly (mean 66 years), frequently those with a history of hypertension (61%), atherosclerotic cardiovascular disease (44%), renal failure (34%), and aortic aneurysm (25%) at presentation.

Clinical presentation varies and depends on the origin of the atheroma

• if the proximal aorta has been disturbed then symptoms may occur in the central nervous system, abdominal organs, and extremities. The lower extremities are involved if the site is below the renal arteries.

Possible features of cholesterol emboli syndrome is characterised include:

• livedo reticularis
  • skin is involved in 35% of patients with CCE. Livedo reticularis is the most common skin disorder (49%), followed by gangrene (35%), cyanosis (28%), and ulceration (17%) (1)
• renal failure
  • typical patient at risk is a man older than 60 years, with a history of hypertension, smoking, and arterial disease
  • in such a patient, the triad of a precipitating event (most common triggering event was coronary angiography via the femoral artery), acute/subacute renal failure, and signs of peripheral emboli strongly suggests the diagnosis (2)
• eosinophilia

Other possible feaures include:

• myalgia
• fever
• trash foot - triad of thigh and foot pain, livedo reticularis, and intact peripheral pulses is considered to be pathognomonic for cholesterol embolisation
• other possible manifestations include transient ischaemic attacks, amaurosis fugax and rarer manifestations such as hypertension, bowel ischaemia and infarction, pancreatitis, and adrenal insufficiency

Diagnosis

• laboratory tests that may aid in the diagnosis of cholesterol emboli syndrome include eosinophilia, eosinophiluria, increased sedimentation rate, and decreased complement concentrations. Final diagnosis is established with biopsy and the aforementioned clinical findings (1)
• note that diagnosis may be difficult because there may be a delay between the precipitating event and the appearance of the syndrome:
  • the typical delay is 1-4 weeks
  • rarely there may be a delay of several months

Reference:

• Heart 2003;89:e17 (http://www.heartjnl.com/cgi/content/full/89/5/e17)
• Scolari F, Ravani P, Gaggi R, et al. (July 2007). The challenge of diagnosing atheroembolic renal disease: clinical features and prognostic factors. Circulation 116 (3): 298–304.