Objective Lipoprotein glomerulopathy (LPG) is a rare inherited renal disease caused by mutation of the APOE gene.It is characterized by proteinuria and nephrotic syndrome with elevated serum apoE.The treatment and outcome of LPG remain unknown.Methods All 35 patients of 31 unrelated Han families were diagnosed as LPG by renal biopsy from the same county in the southwest of China.APOE Kyoto mutation (p.Arg25Cys) was detected in LPG patients and 28 asymptomatic relatives by DNA sequencing.Results All of the mutations shared the same allele ε3.LPG patients demonstrated proteinuria, higher total triglyceride (TG) and higher serum apoE compared with non-carriers.Serum apoE and TG levels of asymptomatic carriers were between LPG patients and non-carriers.Sixteen patients that received treatment of fenofibrate for over 12 months showed clinical improvement.Eight (50%) reached complete remission (proteinuria <1 g/d with stable serum creatinine) with intensive control of their lipid profile (normalized serum apoE and TG<100 mg/dL).Six (37.5%) reached partial remission.At 3-year follow-up, patients receiving treatment of fenofibrate had superior survival and stable renal function.Conclusions To our knowledge, this is the largest series of LPG patients ever reported.Carriers with elevated serum apoE levels and TG may be predisposed to clinical LPG.Fenofibrate can induce the remission of LPG.