AbstractIn this issue of Clinical Kidney Journal, Gamayo et al. describe two cases of anti-low-density lipoprotein receptor-related protein 2 (LRP2) nephropathy. This is a recently described entity that has features of both tubulointerstitial disease and segmental membranous nephropathy. The originality of the present report consists of the association of a disease thought to be rare (only 13 in prior described patients, 11 in the past year) with B-cell lymphoproliferative disease. Together with the finding of a third case among 224 elderly patients studied, this raises the issue of the underdiagnoses of LRP2 nephropathy, on top of the potential association to B-cell malignancy. We now put these findings in context within the wider frame of autoimmunity against megalin/LRP2 and related antigens such as Fx1A and CD69.