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.
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.
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]