J Korean Med Sci.  2009 Jan;24(Suppl 1):S44-S49. 10.3346/jkms.2009.24.S1.S44.

Clinicopathologic Characteristics of IgA Nephropathy with Steroid-responsive Nephrotic Syndrome

• Kim SM
• Moon KC
• Oh KH
• Joo KW
• Kim YS
• Ahn C
• Han JS
• Kim S

• KMID: 1778139
• DOI: http://doi.org/10.3346/jkms.2009.24.S1.S44

Abstract

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 steroidresponsive 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.

Keyword

Glomerulonephritis, IGA; Nephrosis, Lipoid; Nephrotic Syndrome; Steroids

MeSH Terms

Adult
Aged
Female
Glomerulonephritis, IGA/complications/*diagnosis
Humans
Kidney Transplantation
Korea
Male
Microscopy, Fluorescence/methods
Middle Aged
Nephrotic Syndrome/complications/*diagnosis/therapy
Remission Induction
Retrospective Studies
Steroids/*therapeutic use
Treatment Outcome

Reference

1. Mustonen J, Pasternack A, Rantala I. The nephrotic syndrome in IgA glomerulonephritis: response to corticosteroid therapy. Clin Nephrol. 1983. 20:172–176.

2. Wu G, Katz A, Cardella C, Oreopoulos DG. Spontaneous remission of nephrotic syndrome in IgA glomerular disease. Am J Kidney Dis. 1985. 6:96–99.

3. Lai KN, Lai FM, Chan KW, Ho CP, Leung AC, Vallance-Owen J. An overlapping syndrome of IgA nephropathy and lipoid nephrosis. Am J Clin Pathol. 1986. 86:716–723.

4. Sinnassamy P, O'Regan S. Mesangial IgA deposits with steroid responsive nephrotic syndrome: probable minimal lesion nephrosis. Am J Kidney Dis. 1985. 5:267–269.

5. Choi J, Jeong HJ, Lee HY, Kim PK, Lee JS, Han DS. Significance of mesangial IgA deposition in minimal change nephrotic syndrome: a study of 60 cases. Yonsei Med J. 1990. 31:258–263.

6. Matsukura H, Miya K, Arai M, Miyawaki T, Inaba S. Minimal change variants with mesangial IgA deposits. Clin Nephrol. 2007. 68:337–338.

7. Westhoff TH, Waldherr R, Loddenkemper C, Ries W, Zidek W, van der Giet M. Mesangial IgA deposition in minimal change nephrotic syndrome: coincidence of different entities or variant of minimal change disease? Clin Nephrol. 2006. 65:203–207.

8. Lee HS, Lee MS, Lee SM, Lee SY, Lee ES, Lee EY, Park SY, Han JS, Kim S, Lee JS. Histological grading of IgA nephropathy predicting renal outcome: revisiting H. S. Lee's glomerular grading system. Nephrol Dial Transplant. 2005. 20:342–348.

9. Abreo K, Wen SF. A case of IgA nephropathy with an unusual response to corticosteroid and immunosuppressive therapy. Am J Kidney Dis. 1983. 3:54–57.

10. Garcia Camin RM, Cabezas A, Calero F, Ballarin JA. Steroid-responsive nephrotic syndrome in IgA nephropathy. Nefrologia. 2004. 24:Suppl 3. 68–71.

11. Philibert D, Cattran D, Cook T. Clinicopathologic correlation in IgA nephropathy. Semin Nephrol. 2008. 28:10–17.

12. Lai KN, Lai FM, Ho CP, Chan KW. Corticosteroid therapy in IgA nephropathy with nephrotic syndrome: a long-term controlled trial. Clin Nephrol. 1986. 26:174–180.