Yan Hong1,2, Pengxian Shi1, Xia Liu3, Liu Yang3, Kang Li3, Feng Xu3, Shaoshan Liang3, Zhengzhao Liu3, Haitao Zhang3, Yinghua Chen4, Weixin Hu5,6. 1. Jinling Clinical Medical College of Nanjing Medical University, Nanjing, China. 2. The Affiliated Wuxi No. 2 People's Hospital of Nanjing Medical University, Wuxi, China. 3. National Clinical Research Centre of Kidney Diseases, Nanjing Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. 4. National Clinical Research Centre of Kidney Diseases, Nanjing Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. cherry1109@163.com. 5. Jinling Clinical Medical College of Nanjing Medical University, Nanjing, China. huwx@vip.163.com. 6. National Clinical Research Centre of Kidney Diseases, Nanjing Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. huwx@vip.163.com.
Abstract
OBJECTIVES: To retrospectively investigate the clinical and histological features and outcomes of ANCA-associated glomerulonephritis (AAGN) with different ANCA serotypes. METHOD: A total of 467 AAGN patients were divided into MPO-AAGN (MPO) and PR3-AAGN (PR3) groups according to ANCA serotype. Clinical and histological features and renal outcomes were compared. RESULTS: In this study, 429 (91.9%) patients tested positive for MPO-ANCA, and 38 (8.1%) for PR3-ANCA. The median age at diagnosis (P = 0.017) and proportion of females (P = 0.003) were higher in the MPO group. Joint (P < 0.001), ENT (P = 0.000), skin (P = 0.007), and eye (P = 0.014) involvements were more common in the PR3 group. Compared with that in the PR3-group, a higher proportion of patients in the MPO group had microscopic polyangiitis (P = 0.000), and a lower proportion of exhibited granulomatosis with polyangiitis (P = 0.000). Patients in the MPO group also exhibited lower BVAS scores (P = 0.003) and higher serum albumin levels (P = 0.009). Histologically, a lower proportion of MPO patients had crescentic glomerulonephritis (P = 0.028) and acute tubule-interstitial lesion scores (P = 0.007), but a higher proportion of these patients exhibited mixed class glomerulonephritis (P = 0.032) than in the PR3 group. The relapse rate was lower (P = 0.020), and the 5-year relapse-free survival rate (P = 0.003) was higher in the MPO group than in the PR3 group. However, the 5-year renal survival rates (P = 0.106) were not significantly different. CONCLUSIONS: MPO-ANCA was predominant in Chinese patients with ANCA-associated vasculitis and renal disease. The epidemiological characteristics, extra-renal involvement, and histopathological classes and outcomes were different between MPO-positive and PR3-positive patients, implying that they might be two different disease entities.
OBJECTIVES: To retrospectively investigate the clinical and histological features and outcomes of ANCA-associated glomerulonephritis (AAGN) with different ANCA serotypes. METHOD: A total of 467 AAGN patients were divided into MPO-AAGN (MPO) and PR3-AAGN (PR3) groups according to ANCA serotype. Clinical and histological features and renal outcomes were compared. RESULTS: In this study, 429 (91.9%) patients tested positive for MPO-ANCA, and 38 (8.1%) for PR3-ANCA. The median age at diagnosis (P = 0.017) and proportion of females (P = 0.003) were higher in the MPO group. Joint (P < 0.001), ENT (P = 0.000), skin (P = 0.007), and eye (P = 0.014) involvements were more common in the PR3 group. Compared with that in the PR3-group, a higher proportion of patients in the MPO group had microscopic polyangiitis (P = 0.000), and a lower proportion of exhibited granulomatosis with polyangiitis (P = 0.000). Patients in the MPO group also exhibited lower BVAS scores (P = 0.003) and higher serum albumin levels (P = 0.009). Histologically, a lower proportion of MPOpatients had crescentic glomerulonephritis (P = 0.028) and acute tubule-interstitial lesion scores (P = 0.007), but a higher proportion of these patients exhibited mixed class glomerulonephritis (P = 0.032) than in the PR3 group. The relapse rate was lower (P = 0.020), and the 5-year relapse-free survival rate (P = 0.003) was higher in the MPO group than in the PR3 group. However, the 5-year renal survival rates (P = 0.106) were not significantly different. CONCLUSIONS:MPO-ANCA was predominant in Chinese patients with ANCA-associated vasculitis and renal disease. The epidemiological characteristics, extra-renal involvement, and histopathological classes and outcomes were different between MPO-positive and PR3-positive patients, implying that they might be two different disease entities.
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