A case of lipoprotein glomerulopathy in which complete remission was achieved by pemafibrate therapy.

Lipoprotein glomerulopathy (LPG) is a rare hereditary glomerular disease characterized by lipoprotein thrombi within dilated glomerular capillaries for which effective treatments remain limited. This report concerns a 25-year-old Japanese woman with an 8-year history of persistent proteinuria and severe dyslipidemia who presented with heavy proteinuria (3.31 g/gCr) despite preserved renal function. Kidney biopsy revealed features typical of LPG on light and electron microscopy, and serum apoE levels were markedly elevated. The patient was started on pemafibrate, a selective peroxisome proliferator-activated receptor-alpha modulator known to exert stronger triglyceride-lowering effects and to have a more favorable hepatic and renal safety profile compared with conventional fibrates. Following treatment, the patient's triglyceride levels decreased substantially and her proteinuria progressively improved. Complete remission (0.05 g/gCr) was achieved within 7 months, and she remained stable thereafter. Although partial remission with fibrates has been previously reported in patients with LPG, there have been no reports of complete remission in those treated with pemafibrate. To our knowledge, this is the first documented case of complete remission of proteinuria induced by pemafibrate in a patient with LPG. Pemafibrate may be a promising targeted therapy for this rare glomerular disorder.