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[Cholesterol embolism--a serious systemic disease]
E Palmgren1, M Hartford, H Herlitz
1Njurmedicinska avdelningen, Universitetssjukhuset, Sahlgrenska, Göteborg.
Insights
Cholesterol embolism syndrome, a severe condition affecting multiple organs, particularly the kidneys, has a high mortality rate. Early diagnosis and prophylaxis in patients with atherosclerosis undergoing vascular procedures are crucial due to the lack of effective treatments.
Area of Science:
- Nephrology
- Cardiovascular Medicine
- Pathology
Background:
- Cholesterol embolism syndrome (CES) is a serious multiorgan disorder resulting from cholesterol crystal emboli originating from atherosclerotic plaques.
- The kidneys are most frequently affected (approximately 50% of cases), often leading to progressive renal failure.
- CES carries a poor prognosis, with a mortality rate around 70%.
Observation:
- This report details two of four CES cases diagnosed in elderly men with advanced atherosclerosis during autumn 1997.
- Diagnosis was confirmed by identifying cholesterol crystals in renal arteries via biopsy.
- Patients presented with progressive renal failure, initially suspected as vasculitis or radiologically induced renal damage.
Findings:
- Renal biopsy did not reveal vasculitis in any patient initially.
- One patient, initially ANCA positive, later developed vasculitis.
- Three out of four patients died within two months; one patient with renal vasculitis survived for 1.5 years after treatment with cyclophosphamide and steroids.
Implications:
- CES should be strongly considered in elderly patients with atherosclerosis presenting with progressive renal and multi-organ failure.
- Invasive vascular procedures like angiography and surgery increase the risk of CES.
- Prophylaxis, including identifying at-risk patients before procedures, is paramount as no effective therapy currently exists.
Abstract:
The syndrome of cholesterol embolism is a multiorgan disorder caused by peripheral embolization of cholesterol crystals from an ulcerated aorta plaque. The kidney is the organ most often affected (approx 50%). Prognosis is poor, with a mortality of about 70%. We describe two of four cases of cholesterol embolism diagnosed during the autumn of 1997. All were elderly men with advanced generalized atherosclerosis. Positive diagnosis upon renal biopsy required demonstrating cholesterol crystals in renal interlobar and arcuate arteries. Workups for progressive renal failure were performed due to suspected vasculitis in three cases and radiologically induced renal damage subsequent to coronary angiography in another. None of the patients showed renal vasculitis upon biopsy, but one who was ANCA positive developed vasculitis after a few months. Three patients died within a couple of months of onset. The patient with renal vasculitis was treated with cyclophosphamide and steroids, and survives one and a half years after onset. It is vital to consider the diagnosis cholesterol embolism whenever an elderly patient with pronounced atherosclerosis develops progressive renal failure and multi-organ failure. Angiography, aorto-iliaco-femoral surgery and thrombolytic therapy increase the risk of cholesterol embolism in this group. There is no effective therapy. The key is prophylaxis, which means identifying patients at risk prior to invasive vascular procedures.