Lupus podocytopathy: a distinct entity of lupus nephritis.

Systemic lupus erythematosus (SLE) patients clinically presenting with nephrotic syndrome demonstrating minimal change disease (MCD), mesangial proliferation (MsP) or focal segmental glomerulosclerosis (FSGS), while on electronic microscopy, diffuse podocyte foot process effacement in absence of sub-epithelial or sub-endothelial deposition is the only morphological feature and now diagnosed as lupus podocytopathy. Lupus podocytopathy with glomerular morphology of MCD or MsP usually presents with typical nephrotic syndrome and sensitive to glucocorticoid treatment, but the relapse rate could reach up to 90% on maintenance treatment with glucocorticoid alone. Glucocorticoid plus other immunosuppressive agents could significantly decrease the relapse rate. Lupus podocytopathy with FSGS presents with a higher rate of acute kidney injury and less sensitivity to glucocorticoid treatment. The long-term outcomes of lupus podocytopathy are optimistic, but pathological transition could occur after renal relapses. The unique clinical and morphological features of lupus podocytopathy indicate that this special SLE-associated glomerulopathy should be included in the upcoming revised pathological classification of lupus nephritis.