Post-transplantation recurrence of lipoprotein glomerulopathy: report of 4 cases and literature review.

Lipoprotein glomerulopathy (LPG) is an ultra-rare kidney disorder caused by pathogenic variants in the APOE gene. Although kidney biopsy presents typical findings, such as dilated capillary loops containing lipoprotein thrombi, definitive diagnosis requires molecular genetic analysis of APOE. There is no specific treatment for the disease and, in the scenario of a disorder with glomerular lipoprotein deposition, the disease may recur after kidney transplantation. Herein we report four cases of post-transplantation recurrence of LPG in Brazilian patients, including one case of early relapse (in the first year following transplantation) and three cases of late relapse. Two of the patients had the APOE Kyoto variant while two harbored the APOE Osaka/Kurashiki variant. As in previously described cases, the clinical response was heterogeneous despite the use of statins and antiproteinuric agents, including remission or persistence of proteinuria and progression to different stages of chronic kidney disease. Such cases strongly support molecular genetic investigation of cases with suspicion of LPG, even in a Latin American population, since a confirmed diagnosis raises the possibility of disease recurrence in the kidney graft and can provide valuable information for selecting a potential living kidney donor.