Renal-limited 'lupus-like' Nephritis

In the setting of an IgG-dominant immune complex-mediated glomerulonephritis, there are multiple pathological findings that strongly suggest the diagnosis of lupus nephritis (LN) including (i) 'full-house' immunofluorescence staining for IgG, IgM, IgA, C3 and C1; (ii) extraglomerular immune deposits; (iii) combined mesangial, subendothelial and subepithelial immune deposits and (iv) the presence of endothelial tubuloreticular inclusions. We report four female adult patients with renal biopsy findings which are highly suggestive of LN but without extrarenal signs, symptoms or serologies of systemic lupus erythematosus at the time of biopsy or over a mean follow-up period of 3 years. Despite aggressive therapy, outcomes were poor in this small cohort. We refer to these cases as renal-limited 'lupus-like' nephritis.