Clinicopathologic characteristics of IgA nephropathy with steroid-responsive nephrotic syndrome.
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.
Kim
Sun Moon
SM
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
Moon
Kyung Chul
KC
Oh
Kook-Hwan
KH
Joo
Kwon Wook
KW
Kim
Yon Su
YS
Ahn
Curie
C
Han
Jin Suk
JS
Kim
Suhnggwon
S
D000328:Adult; D000368:Aged; D005260:Female; D005922:Glomerulonephritis, IGA / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D016030:Kidney Transplantation; D007723:Korea; D008297:Male; D008856:Microscopy, Fluorescence / Q000379:methods; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D012074:Remission Induction; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D013256:Steroids