LAUSR

INTRODUCTION L ipoprotein glomerulopathy (LPG) is caused by mutations in APOE, the gene encoding apolipoprotein E. Themost commonly encounteredmutations are APOE Sendai (p.Arg145Cys),APOEKyoto (p.Arg25Cys or p.Arg43Cys), and APOE Tokyo (p.Leu141_Lys143del or p.Arg142_Leu144del), whose names correspond to the cities in which the index patients lived. Most cases have been reported from East Asian countries; only a few cases from the United States have been described.We present a case of LPG that was first diagnosed in a protocol kidney allograft biopsy. Diagnosis was challenging because of the many unusual features, including the patient’s European ancestry, lack of family history or significant proteinuria, very young age at progression to chronic renal failure, which had been erroneously attributed to primary membranoproliferative glomerulonephritis (MPGN), and complex kidney allograft histologic features consisting of donor-derived and subsequent posttransplant complications.