Goodpasture's syndrome with concomitant immune complex mixed membranous and proliferative glomerulonephritis

Classical Goodpasture's (GP) syndrome is a monophasic illness characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis with linear IgG deposition along the glomerular and distal tubular basement membrane and destructive necrotizing diffuse extracapillary crescentic glomerulonephritis. The majority of patients have circulating anti-glomerular basement membrane (GBM) antibodies, detectable with standard anti-GBM ELISA. Concurrence of GP syndrome with proliferative glomerulonephritis has only rarely been described. In this report, for the first time we describe in a 21-year-old woman GP syndrome with 50% crescentic sclerosing glomerulonephritis with linear immunofluorescence characteristic of anti-GBM pathogenesis, combined with mixed membranous and membranoproliferative glomerulonephritis with granular immunofluorescence and subepithelial, mesangial and subendothelial deposits characterizing immune complex pathogenesis. The clinical picture was also unusual for GP syndrome, manifesting a recurrent but non-progressive course, nephrotic syndrome, normal renal function and low values of anti-GBM antibodies, identified only by novel more sensitive techniques

Mean urine IgG to albumin ratio in responders (R) and non-responders (NR) in time.

<p>Mean urine IgG to albumin ratio in responders (R) and non-responders (NR) in time.</p

ROC curve with AUC for urine IgG to albumin ratio at the time of diagnosis.

<p>ROC curve with AUC for urine IgG to albumin ratio at the time of diagnosis.</p

Mean urine protein to creatinine ratio in responders (R) and non-responders (NR) in time.

<p>Mean urine protein to creatinine ratio in responders (R) and non-responders (NR) in time.</p

Mean of NAG to creatinine ratio in responders (R) and non-responders (NR) in time.

<p>Mean of NAG to creatinine ratio in responders (R) and non-responders (NR) in time.</p

ROC curve with AUC for urine NAG to creatinine ratio at the time of diagnosis.

<p>ROC curve with AUC for urine NAG to creatinine ratio at the time of diagnosis.</p