Background: Patients with nephrotic syndrome have impaired endothelial function probably related to dyslipidemia. This study evaluated the effects of statin therapy on dyslipidemia and endothelial function in patients with nephrotic syndrome. Methods: A sequential, open-label study of the effects of statins on endothelial dysfunction in 10 nephrotic patients treated with an angiotensin-converting enzyme (ACE) inhibitor or angiotensin II (Ang II) receptor antagonist. Endothelial function was assessed at baseline, after 12 weeks of treatment with statins, and after an 8-week washout. Brachial artery endothelial function was measured as post-ischemic flow-mediated dilation (FMD) using ultrasonography. Endothelium-independent, glyceryl trinitrate-mediated vasodilation (GTNMD) also was measured. Results: Serum lipids were significantly lower following statin: total cholesterol mean 8.2 ± 0.4 (standard error) mmol/L versus 5.2 ± 0.3 mmol/L, triglycerides 2.6 ± 0.4 mmol/L versus 1.6 ± 0.2 mmol/L, non-HDL-cholesterol 6.7 ± 0.4 mmol/L versus 3.7 ± 0.2 mmol/L (all P