Peer-review started: February 12, 2019
First decision: March 15, 2019
Revised: April 1, 2019
Accepted: April 8, 2019
Article in press: April 8, 2019
Published online: June 28, 2019
Processing time: 140 Days and 12.9 Hours
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.
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.
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.
Core tip: Spontaneous atheroembolic renal disease (AERD) is a rare clinical entity. The role of antineutrophil cytoplasmic antibodies (ANCA) in atheroembolic diseases remains to be elucidated. Here, we report a case of rapidly progressive renal failure initially managed as cytoplasmic-ANCA associated renal disease but subsequently diagnosed as AERD with renal biopsy that responded surprisingly well to steroid therapy in a 62-year-old female patient. The patient was discharged with oral prednisone, and her renal function continued to improve during the initial follow-up. Although no specific treatment is available, the potential role of steroids requires exploration.