A rare presentation of spontaneous atheroembolic renal disease: A case report

Abstract

Background: Atheroembolic renal disease (AERD) is caused by occlusion of the small renal arteries from embolized cholesterol crystals arising from ulcerated atherosclerotic plaques. This usually manifests as isolated renal disease or involvement from systemic atheroembolic disease. Here we report a case of AERD that responded well to steroid therapy.

Case summary: A 62-year-old woman with a history of hypertension and stage IIIa chronic kidney disease was referred for rapidly worsening renal function over a 4-mo period. She complained of swollen legs, dyspnea on exertion, and two episodes of epistaxis about a month prior to admission. She reported no history of invasive vascular procedures, use of radio contrast agents, or treatment with anticoagulants or thrombolytic agents. Urinalysis showed a few red blood cells and granular casts. Serology was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Non-contrast-enhanced computed tomography of the chest, abdomen, and pelvis showed diffuse atherosclerotic changes in the aortic arch. Thus, c-ANCA-associated vasculitis was suspected, and the patient was started on pulse intravenous methylprednisolone. Her renal biopsy showed evidence of AERD. She was discharged with oral prednisone, and her renal function continued to improve during the initial follow-up.

Conclusion: In cases of non-vasculitis-associated ANCA, a high degree of clinical suspicion is required to pursue the diagnosis of spontaneous AERD in patients with clinical or radiological evidence of atherosclerotic burden. Although no specific treatment is available, the potential role of statins and steroids requires exploration.

Keywords: Antineutrophil cytoplasmic antibodies associated vasculitis; Atheroembolic renal disease; Case report; Chronic kidney disease.

Figure 1

Atheroembolic disease of the kidney. A, B: Several small arteries and arterioles are affected and show needle-shaped clefts in occluded and recanalized vessels (see arrow).

Figure 2

Focal global and segmental glomerulosclerosis (50% of glomeruli). Arrow: Acute tubular injury, mild.

Figure 3

Electron microscopy showing sparsely distributed mesangial and glomerular capillary wall deposits (electron dense) (see arrow).

Figure 4

Ultra structural features suggestive of a mild and currently quiescent or inactive IgA nephropathy or sequelae of an IgA-dominant infection-associated glomerulonephritis. There are sparsely distributed mesangial and glomerular capillary wall deposits reactive for IgA, IgM, C3, and with equal expression of kappa and lambda light chains. There are no signs of a currently active glomerulitis. A: Mild IgA deposition; B: IgG deposition; C: IgM deposition; D: C3 deposition; E: Kappa and lambda chain deposition; F: Fibrin deposition.