Nephrotic syndrome is an unusual manifestation of IgA Nephropathy (IgAN). Some 
cases respond to steroid treatment. Here we describe a case-series of IgAN 
patients with steroid-responsive nephrotic syndrome. Twelve patients with IgAN 
with steroid-responsive nephrotic syndrome were evaluated and followed up. All 
patients presented with generalized edema. Renal insufficiency was found in two 
patients. The renal biopsy of eight patients revealed wide foot process 
effacement in addition to the typical features of IgAN. They showed complete 
remission after steroid therapy. Seven relapses were reported in five patients; 
six of the relapsed cases responded to steroid therapy. Compared with 
steroid-non-responsive patients, the patients with steroid-responsive nephrotic 
syndrome had shorter symptom duration, more weight gain, more proteinuria, and 
lower histologic grade than did those that had steroid-non-responsive nephrotic 
syndrome at presentation. None of the responders progressed to end stage renal 
disease, whereas five (38%) non-responders required dialysis or renal 
transplantation. Patients with IgAN who have steroid-responsive nephrotic 
syndrome likely have both minimal change disease and IgAN. The clinical features 
of sudden onset of generalized edema, initial heavy proteinuria and initial 
severe hypoalbuminemia might help identify the subset of patients, especially in 
low grade IgAN.