Clinicopathological characteristics and predictors of renal outcomes in diffuse crescentic glomerulonephritis : a retrospective single-center study from Western China study

Abstract

Abstract Aim: To investigate the clinicopathological characteristics and prognosis among various types of diffuse crescentic glomerulonephritis(DCGN) and especially type II DCGN and to identify predictive factors for renal outcomes in these patients. Methods: This study retrospectively examined 107 patients diagnosed with diffuse crescentic glomerulonephritis through biopsy at the Sichuan Provincial People's Hospital, spanning from January 2012 to July 2022. The investigation primarily aimed to identify the occurrence of end-stage kidney disease (ESKD) as the primary endpoint. Analytical methods included Cox regression models and Kaplan-Meier survival analysis to assess the data. Results: Among the 107 enrolled patients, 12 (11.2%) patients had Type I diffuse crescentic glomerulonephritis, 70 (65.4%) patients had Type II (including immunoglobulin A [IgA] nephropathy, 40%; lupus nephritis, 38.6%), and 25 (23.4%) patients had Type III. Thirty-seven (34.6%) patients received kidney replacement therapy (KRT) at presentation, while 17 (15.9%) received plasmapheresis. In the follow-up cohort, 46 (47%) patients progressed to ESKD. For patients with diffuse crescentic glomerulonephritis (DCGN), the 5-year cumulative renal survival rates were markedly different across the three types: Type I had a rate of 0%, Type II had a rate of 57.5%, and Type III had a rate of 18.6%. Furthermore, among Type II patients, IgA nephropathy emerged as the most prevalent condition. The cumulative 5-year kidney survival rates were 50% for patients with IgA nephropathy, 64% for lupus nephritis, and 70% for Henoch-Schönlein purpura nephritis. A significant association between the risk of ESKD development and several factors was revealed by a multivariate Cox regression analysis: baseline serum creatinine level (P=0.001), initial kidney replacement therapy (KRT) at presentation (P=0.003), interstitial inflammation (P=0.023), global glomerulosclerosis (P=0.009), and the presence of fibrous crescents over 50% (P=0.033). Conclusion: Type II diffuse crescentic glomerulonephritis was the most prevalent type in DCGN, and favors better renal prognosis than type I and III DCGN, in which IgA nephropathy was the most common entity of Type II DCGN in western China. The prognosis for IgA nephropathy was worse than that for patients with lupus nephritis or Henoch-Schönlein purpura nephritis. Additionally, baseline serum creatinine level, initial KRT at presentation, interstitial inflammation, global glomerulosclerosis, and fibrous crescents >50% were identified as predictors of renal outcomes in diffuse crescentic glomerulonephritis .