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

Lipoprotein glomerulopathy (LPG) is an ultrarare 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, it may recur after kidney transplantation. Herein we reported 4 cases of post-transplant recurrence of LPG in Brazilian patients, including 1 case of early relapse (in the first year following transplantation) and 3 cases of late relapse. Two of the patients had the APOE Kyoto variant, while 2 harbored the APOE Osaka/Kurashiki variant. As in previously described cases, the clinical response was heterogeneous despite the use of statins and antiproteinuric agents, with proteinuria remission or persistence and progression to different stages of chronic kidney disease. Such cases strongly support the molecular genetic investigation of cases suspected of LPG, even in a Latin American population. A confirmed diagnosis can raise the likelihood of disease recurrence in the kidney graft and provide valuable information for selecting a potential living kidney donor.