Bilateral Renal Artery Involvement of Erdheim-Chester Disease

To report a rare case of nonatherosclerotic renal artery disease (NARAD) in a patient with Erdheim-Chester disease (ECD), a non-Langerhans cell histiocytosis. The patient is a 75-year-old male who presented with 2 years of hypertension secondary to right renal artery occlusion incidentally found on cardiac catheterization. Open bilateral renal revascularization was performed because of improved long-term graft patency demonstrated in other forms of NARAD. Nine months postoperatively, the patient's hypertension was significantly improved and surveillance duplex demonstrated patent bilateral renal artery bypasses. Revascularization in patients with NARAD, such as ECD, should be managed in an open approach because of the superior long-term graft patency and blood pressure control in a disease where progression to vessel fibrosis and atherosclerosis could interfere with endovascular stent patency.