NDT Advance Access originally published online on November 28, 2007
Nephrology Dialysis Transplantation 2008 23(4):1298-1306; doi:10.1093/ndt/gfm775
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The prognosis and pathogenesis of severe lupus glomerulonephritis
1 The Department of Pathology 2 The Section of Nephrology, Department of Medicine, Rush University Medical College, Chicago, IL 60612, USA
Melvin M. Schwartz, Department of Pathology, Rush University Medical College, 1753 W. Congress Parkway, Chicago, IL 60612, USA. Tel: +1-312-942-5262; Fax: +1-312-942-4228; E-mail: Melvin_Schwartz{at}rush.edu
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Abstract |
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Background. The International Society of Nephrology/ Renal Pathology Society classification (ISN/RPS) of lupus glomerulonephritis (GN) divides diffuse GN (
50% involvement) into diffuse segmental (IV-S) and diffuse global GN (IV-G). This division tests whether the pathogenesis and clinical outcomes are the same as when similar patients are classified using the World Health Organization (WHO) classification into severe segmental (WHO III 50%) and diffuse global (WHO-IV) GN.
Methods. Thirty-nine renal biopsies with WHO class IV and 44 with WHO III 50% were reclassified using the ISN/RPS and were correlated with pathogenesis and outcome.
Results. There were 22 biopsies with ISN/RPS class IV-S. ISN/RPS class IV-G comprises two morphologically discrete classes of renal biopsies: 39 biopsies originally classified as WHO class IV (WHO-IV) and 22 that switched from WHO III 50% to ISN/RPS class IV-G (IV-Q). We will analyze IV-S, IV-Q and WHO-IV separately. WHO-IV had significantly more immune aggregate deposition than IV-S and IV-Q. WHO-IV had lower serum complements C3 (P = 0.05) and C4 (P = 0.05) than patients with IV-Q. Patients with WHO-IV had more remissions (56%) than IV-Q (23%) (P = 0.01), and stable renal function at the last follow-up was less frequent in patients with IV-Q (18%) than IV-S (50%, P = 0.05) and WHO-IV (62%, P = 0.001). Renal survival and renal survival without end-stage renal disease were different when the patients were diagnosed as WHO classes III 50% and IV, but the outcomes for ISN/RPS class IV-S and IV-G (WHO-IV plus IV-Q) were not different.
Conclusions. WHO III 50% and WHO-IV lupus GN are not congruent with ISN/RPS IV-S and IV-G. The ISN/RPS minimizes pathological and outcome differences between classes IV-S and IV-G which results in the loss of informational content from the renal biopsies. ISN/RPS does not detect pathogenetic or clinical differences among patients with severe lupus GN.
Keywords: diffuse global glomerulonephritis; hyaline thrombi; pathology; patient survival; remissions; renal survival; wire loops; subendothelial deposits; IV-S; IV-G
3 See the appendix for the members of the Collaborative Study Group.
Received for publication: 29. 6.07
Accepted in revised form: 4.10.07
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