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Background: Hypertension is common in the antiphospholipid (Hughes) syndrome (APS) and its cause is poorly understood. Anecdotal evidence suggests that renal artery stenosis (RAS) may be a relevant and treatable cause of hypertension.Objective: To investigate the prevalence of RAS in patients with APS and hypertension.Patients and Methods: Three groups of patients were evaluated: (1) 77 patients with positive antiphospholipid antibodies (aPL) (60 secondary APS, 11 primary APS, and 6 with aPL only) and uncontrolled hypertension who were receiving two or more antihypertensive drugs; (2) 91 patients (Results: Group 1: 20/77 (26%) patients had evidence of RAS (16 unilateral and 4 bilateral). Sixteen patients (80%) had smooth well defined stenoses in the proximal third of the renal artery. Three further patients had irregular arteries without distinct stenosis. Group 2: 7/91 (8%) hypertensive patients had RAS (chi(2)=10.3, p<0.001 v group 1). Group 3: 3/92 (3%) healthy donors had RAS (chi(2)=18.2, p<0.0001 v group 1).Conclusion: A significantly increased prevalence of RAS (26%) was found in patients with APS and hypertension, compared with relatively young (