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TomPettersson

Cholesterol Embolization

Essentials

  • In cholesterol embolization syndrome cholesterol crystals are released from the arterial wall and dispersed in the circulation, causing ischaemic and inflammatory damage in the target organ and a syndrome mimicking systemic vasculitis.
  • Cholesterol embolization has to be taken into account as a cause of vascular occlusive or systemic symptoms, particularly if a patient with atherosclerosis has undergone angiography or aortic surgery.

Epidemiology

  • Symptomatic cholesterol embolization is a rare, but most likely underdiagnosed pathological condition.
  • Most patients are elderly men with risk factors for cardiovascular disease. Atherosclerosis is the most important risk factor for cholesterol embolization.
  • There is often a preceding interventive diagnostic or therapeutic procedure, e.g. angiography or aortic surgery.
  • Anticoagulant therapy is regarded as a risk factor for cholesterol embolization.

Symptoms and signs

  • Cholesterol embolization is a subacute or chronic pathological condition with slowly developing organ dysfunction.
  • The clinical picture is highly variable and depends on the extent of dissemination of cholesterol emboli.
  • General symptoms
    • Fever
    • Fatigue
    • Lack of appetite
    • Weight loss
  • Skin symptoms
    • Blue toes, distal ulcers and gangrenes
    • The peripheral pulses can usually be felt.
    • Purpura, livedo reticularis
  • Renal symptoms
  • Amaurosis fugax and other transient neurological symptoms
  • Myocardial infarction
  • Intestinal haemorrhage
  • Pancreatitis
  • Myalgia
  • In a hospital-based patient population, mortality approaches 80%.

Laboratory findings

  • Elevated ESR and high serum CRP level in over 80% of patients
  • Leucocytosis in about 40%
  • Eosinophilia in about 80%
  • Thrombocytopenia
  • Hypocomplementaemia

Diagnosis

  • Histological evidence of cholesterol crystals in a biopsy specimen.
  • Ophthalmoscopy may reveal cholesterol emboli in retinal vessels.
  • Clinical diagnosis of cholesterol embolization is possible, if typical symptoms develop after an arterial intervention.
  • Many milder cases probably remain undiagnosed.

Differential diagnosis

  • Cholesterol embolization should be distinguished from atherothrombosis in which the symptoms appear rapidly and are caused by ischaemia or infarction in the target organ.
  • Cholesterol embolization syndrome is a "great masquerader" the clinical manifestation of which can resemble that of systemic vasculitis Vasculitides. Other conditions to be taken into account in differential diagnosis include Raynaud's phenomenon Raynaud's Phenomenon (RP) or White Finger Disease, endocarditis Infective Endocarditis, cardiac myxoma, antiphospholipid antibody syndrome Systemic Lupus Erythematosus (Sle) and thrombotic microangiopathy.
  • Recognition of cholesterol embolization syndrome is important in order to avoid unnecessary immunosuppressive treatment.

Therapy

  • Since cholesterol embolization is a symptom of advanced atherosclerosis, prophylactic therapy for cardiovascular diseases is particularly important: management of weight, blood pressure and blood sugar, smoking cessation, as well as aspirin (ASA) and statin therapy.
  • Surgical treatment may be considered as a life-saving procedure, if the source of embolization can be accurately located.
  • Due to the inflammatory nature of the disease, interleukin 1 inhibitor medication has been considered promising, but experiences are scarce for the time being.

    References

    • Ozkok A. Cholesterol-embolization syndrome: current perspectives. Vasc Health Risk Manag 2019;15():209-220. [PubMed]

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