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We report a case of a patient with cholesterol embolism who showed positive for both myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) and proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) and who was treated with prednisolone (PSL) and cyclophosphamide.
A 50 year old man underwent cardiac catheterisation for back pain. The examination disclosed 90% stenosis of the right coronary artery and a saccular aneurysm in the thoracic aorta. The patient underwent percutaneous transluminal coronary angioplasty and the aneurysm was wrapped with an artificial blood vessel. Postoperatively, the patient had a fever, pleural effusion, abdominal pain, and increased white blood cell (WBC) count, C reactive protein (CRP), and serum creatinine. Cultures of blood and pleural effusion exudate were negative. PSL 15 mg/day was started. However, acute progression of renal failure required haemodialysis.
The patient was transferred to our hospital. Physical examination showed a temperature of 38.0°C and blood pressure of 178/98 mmHg. Cyanosis was noted in both heels and all toes with necrosis and ulcers at the tips of the fifth toes. He had an increased erythrocyte sedimentation rate (ESR) of 82 mm/1st h. Anaemia was noted with a red blood cell count of 2500×109/l, while the patient's WBC count was high at 12×109/l. His platelet count (304×109/l) was within the normal range. Biochemistry showed high levels of blood urea nitrogen (10.0 mmol/l of urea), creatinine (710 μmol/l), and CRP (11.3 mg/l). Complements components were within normal ranges. PR3-ANCA and MPO-ANCA were high at 82E and 29E, respectively.
After admission to hospital, circulatory disturbance in his toes worsened. A diagnosis of ANCA associated vasculitis was made based on systemic inflammatory findings and high levels of WBC, CRP, PR3-ANCA, and MPO-ANCA. High dose steroid treatment was started. Biopsies of the right heel skin and thigh quadriceps showed cholesterol embolism (fig1). However, PSL treatment was continued together with three courses of cyclophosphamide pulse treatment because of persistent fever and high ANCA values. The treatment reduced the fever and toe necrosis, and the ulcers improved. ANCA gradually decreased to normal. The PSL dosage was reduced to 15 mg/day and the patient was discharged.
Cholesterol embolism predominantly affects elderly men with a history of hypertension, atherosclerotic vascular diseases, and renal insufficiency at the time of diagnosis. At least 31% of patients had a preceding history of anticoagulant use or the antecedent performance of a vascular procedure affecting the arterial circulation.1The presence of these cholesterol embolisms within the vascular lumen triggers a characteristic localised inflammatory and endothelial vascular reaction. The inflammatory changes resulting from cholesterol embolism may be responsible for many of the systemic manifestations such as fever, weight loss, myalgias, leucocytosis, eosinophilia, and a raised ESR. Thus cholesterol embolism is referred to as both vasculitis look-alikes2 and pseudovasculitic syndrome.3 The prognosis is poor, particularly in the presence of acute renal failure.4
Three ANCA positive cases5 6 of cholesterol embolism have been described. Peat and Mathieson reported an ANCA positive patient with dyspnoea and haemoptysis after acute deterioration of renal function.5 Cyclophosphamide and PSL improved the symptoms, but cyclophosphamide was discontinued and the PSL dose was reduced because renal and skin biopsies showed cholesterol embolisms. Subsequently, the patient died of intractable cardiac failure.
Kaplan-Pavlovcic et al reported two cases of renal failure with positive MPO-ANCA.6 The details are unknown for one patient. The other patient was treated with corticosteroid alone. This patient required haemodialysis and amputation of the toes. Although their treatment did not result in the improvement of vasculitis, the combination of PSL and cyclophosphamide was effective in our patient with ANCA.
This result suggests that active treatment with corticosteroid and cyclophosphamide should be considered in ANCA positive cases of cholesterol embolism.
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