Rheumatology International

, Volume 35, Issue 4, pp 709–717 | Cite as

Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis

  • Shasha Chen
  • Hao Chen
  • Zhengzhao Liu
  • Haitao Zhang
  • Weixin Hu
  • Zheng TangEmail author
  • Zhihong Liu
Original Article - Observational Research


The objectives of the study were to investigate the pathological features and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. One hundred and one cases of biopsy-proven severe LN with rapidly progressive glomerulonephritis (RPGN) were analyzed in this retrospective study. Another 200 severe LN patients without RPGN were randomly enrolled as a control group. Their clinicopathological data and long-term outcome were compared. There were 76 females and 25 males with an average age of 31.9 ± 14.2 years followed for a median period of 4 years. Compared with controls, patients with RPGN had shorter LN duration (p = 0.008), higher level of creatinine (p < 0.001), severe anemia (p = 0.037), heavier hematuria (p < 0.001), severe tubular injury parameters [NAG (p < 0.001), RBP (p < 0.001), C3 (p < 0.001)], higher scores of AI (p = 0.001) and CI (p = 0.004), higher proportions of glomerular sclerosis (0.033) and crescents (p < 0.001), severe tubulointerstitial lesions (p < 0.001) and interstitial inflammation (p < 0.001), lower rate of complete remission (33.9 vs 68.2 %) and higher rate of treatment failure (46.8 vs 7.9 %). The 3-, 5- and 10-year cumulative renal survival rates of RPGN and non-RPGN patients were 65.1 versus 53.9 versus 42.9 and 96.9 versus 94.9 versus 91.7 %, respectively. Multivariate analysis revealed that SCr concentration and the proportion of crescents were the most important risk factors for end-stage renal disease (ESRD) in severe LN with RPGN (p < 0.001). In conclusion, RPGN occurred in 3.6 % of LN and is associated with severe renal manifestations, serious sclerotic and crescentic glomeruli lesions, severe tubulointerstitial inflammation, atrophy and fibrosis, prominent leukocyte infiltration and worse treatment response. Multivariate analysis revealed that SCr concentration and the proportion of crescents were the most important risk factors for ESRD. 57.1 % of severe LN patients with RPGN might progress to ESRD within 10 years.


Crescent Lupus nephritis Rapidly progressive glomerulonephritis (RPGN) Renal survival Risk factors 


Conflict of interest

We declare that we have no conflict of interest.

Supplementary material

296_2014_3140_MOESM1_ESM.doc (270 kb)
Supplementary material 1 (DOC 270 kb)


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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Shasha Chen
    • 1
  • Hao Chen
    • 1
  • Zhengzhao Liu
    • 1
  • Haitao Zhang
    • 1
  • Weixin Hu
    • 1
  • Zheng Tang
    • 1
    Email author
  • Zhihong Liu
    • 1
  1. 1.National Clinical Research Centre of Kidney Desease, Jinling HospitalNanjing University School of MedicineNanjingChina

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