Nephrotic syndrome in the setting of LECT2 amyloidosis: Take a look at the podocyte.

Amyloid light-chain (AL) amyloidosis represents the most common type of amyloid affecting the kidneys. As AL amyloidosis is frequently clinically manifested as nephrotic syndrome, this glomerular syndrome has been improperly linked to all other types of kidney amyloidosis. In this report, we highlight the importance of amyloid typing, as the deposition of several amyloidotic proteins in the kidneys is not associated with heavy proteinuria. We present two cases of patients who presented with sudden-onset nephrotic syndrome and kidney biopsies showing interstitial, vascular, and/or mesangial LECT2 amyloidosis. Further examination by electron microscopy demonstrated diffuse foot process effacement consistent with minimal change disease and no amyloid deposition in the glomerular basement membrane. Both patients had complete remission after glucocorticoid treatment. We conclude that the presence of nephrotic syndrome in a patient with LECT2 amyloidosis must alert for a potential concurrent podocytopathy.