Renal atheroembolic disease: the Cinderella of nephrology?

Abstract

Atheroembolic renal disease is the Cinderella of nephrology in that often Cinderella (the patient and their clinical findings) and her shoe (the final diagnosis of atheroembolic disease) are not matched in life. Why is this and is it important? There are two major reasons that the diagnosis may not be made. The first is that the condition may mimic many other causes of renal impairment. Some of the features such as livedo reticularis [1], purpuric rash [1], severe hypertension [2,3], hypocomplementaemia [4], raised erythrocyte sedimentation rate [1], eosinophilia [4], or eosinophiluria [5,6], and even a necrotizing glomerulonephritis [7] may make a diagnosis of systemic vasculitis likely [1,8]. The characteristic presentation might be thought of as a sudden and catastrophic decline in function associated with systemic and skin features of atheroembolism [9]. A different and insidious presentation is now recognized with progressive renal dysfunction over a period of months [10,11]. In one series of patients over the age of 65 years biopsied for gradually progressive loss of renal function, atheroembolic disease accounted for 10% of all cases [10]. It is difficult to explain this insidious loss of function by purely mechanical occlusion. A common histological finding is the associated thrombosis seen in the vessels around the crystals [12]. Cholesterol crystals can activate complement and aggregate platelets in vitro and this may explain the progressive nature of the renal dysfunction [13]. In many cases the diagnosis of atheroembolic disease is not made premortem [1] and even if suspected the diagnosis may be made only on clinical suspicion without histological proof for reasons discussed below.