Mimics of Vasculitis

Ernest Maningding; Tanaz A. Kermani


Rheumatology. 2021;60(1):34-47. 

In This Article

Cholesterol Embolization Syndrome (CES)

CES occurs when cholesterol crystals within atherosclerotic plaques embolize to downstream arteries, leading to end-organ damage through mechanical obstruction and/or inflammation. Risk factors include traditional cardiovascular risk factors and a recent procedure involving the aorta and its branches.[84–88] Common sources of cholesterol emboli are the aorta and iliac arteries followed by the femoral arteries.[86] The manifestations of CES can resemble vasculitis with involvement of the skin (livedo reticularis, purpura, nodules, blue toe/ischaemia), kidneys (acute kidney injury, uncontrolled hypertension), gastrointestinal tract (mesenteric ischaemia), CNS (transient ischaemic attack, confusion, amaurosis fugax, stroke) and eyes (involvement of retinal vessels, i.e. Hollenhorst plaques) (Table 1).[84,86–88] Constitutional symptoms and elevated inflammatory markers can also be present. Laboratory abnormalities include anaemia, thrombocytopenia, leucocytosis (eosinophilia), elevated ESR, elevated CRP, elevated fibrinogen, hypocomplementemia, azotaemia, proteinuria, eosinophiluria.[85–88] Definitive diagnosis requires biopsy of the affected tissues (e.g. skin, kidneys, muscle), with histopathology showing lance-shaped clefts (due to dissolution of cholesterol crystals during formalin fixation) occluding the small arteries and arterioles.[86–88]