Predictors for Outcomes in Patients with Severe ANCA-associated Glomerulonephritis who were Dialysis-dependent at Presentation: A Study of 89 Cases in a Single Chinese Center

Objective: Anti-neutrophilcytoplasmic autoantibody (ANCA)-associated vasculitis may cause rapid deterioration of renal function, resulting in high prevalence of end-stage renal disease and mortality. The current study investigated factors associated with restoration of renal function and early mortality in patients with severe ANCA-associated glomerulonephritis, i.e. dialysis-dependent at presentation, in a single Chinese cohort. Methods: Eighty-nine Chinese patients with ANCA-associated glomerulonephritis who were on dialysis at the time of diagnosis were included in this study. All these patients received immunosuppressive therapy plus intravenous methylprednisolone, plasma exchange, or both. The predictive value of the clinical and histological parameters for renal and patient outcome was analyzed. Result: On the sixth month, 25 (28.1%) patients achieved dialysis independence, 45 (50.6%) patients progressed to end stage renal disease, and 19 (21.3%) patients died. Nine out of the 19 deaths were therapy-related. Factors independently associated with renal function restoration were percentages of normal glomeruli (P < 0.05), extent of tubular atrophy (P < 0.05) and extent of interstitial fibrosis (P < 0.05) in the renal specimens. Age and pulmonary hemorrhage were independently associated with all-cause death (P = 0.003 and P = 0.007, respectively) and therapy-related death (P = 0.037 and P = 0.043, respectively). Conclusions: Among patients with severe ANCA-associated glomerulonephritis who were dialysis-dependent at presentation, those with a higher percentage of normal glomeruli and less extent of tubular atrophy/interstitial fibrosis have more chance of restoration of renal function. Increased risk for all-cause death and therapy-related death appears to be older age and pulmonary hemorrhage. (C) 2013 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 42:515-521