Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Nephrol. Jun 28, 2019; 8(3): 67-74
Published online Jun 28, 2019. doi: 10.5527/wjn.v8.i3.67
A rare presentation of spontaneous atheroembolic renal disease: A case report
Paramarajan Piranavan, Ashna Rajan, Vishal Jindal, Ashish Verma
Paramarajan Piranavan, Ashna Rajan, Vishal Jindal, Department of Medicine, Saint Vincent Hospital, Worcester, MA 01608, United States
Ashish Verma, Division of Nephrology, Saint Vincent Hospital, Worcester, MA 01608, United States
Author contributions: Piranavan P, Rajan A, and Verma A provided direct patient care, collected data, and performed follow-up. Piranavan P and Rajan A performed the literature review and wrote the manuscript. Verma A and Vishal Jindal helped locate the relevant literature and draft the manuscript. All authors read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this case report and any accompanying histological images. A copy of the written consent is available for the review of the journal’s editor-in-chief.
Conflict-of-interest statement: The authors declare that they have no competing interests or relevant financial relationships with either individuals or organizations.
CARE Checklist (2016) statement: The manuscript was checked according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Paramarajan Piranavan, MD, Doctor, Resident (PGY3), Department of Medicine, Saint Vincent Hospital, 123, Summer Street, Worcester, MA 01608, United States. paramaraja.piranvan@stivincenthospital.com
Telephone: +1-508-3635000 Fax: +1-508-3639798
Received: February 8, 2019
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
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: Atheroembolic renal disease; Antineutrophil cytoplasmic antibodies associated vasculitis; Chronic kidney disease; Case report

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.