Improved outcome in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis: a 30-year follow-up study

• Published in: Nephrology, dialysis, transplantation Vol. 28; no. 2; pp. 373 - 379
• Main Authors: Hilhorst, Marc, Wilde, Benjamin, van Paassen, Pieter, Winkens, Bjorn, van Breda Vriesman, Peter, Cohen Tervaert, Jan Willem
• Format: Journal Article
• Language: English
• Published: England 01.02.2013
• Subjects: Adult; Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - diagnosis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - epidemiology; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - mortality; Antibodies; antineutrophil cytoplasmic antibodies; Biopsy; Cohort Studies; Comorbidity; Creatinine; Disease Management; Early Diagnosis; Female; Follow-Up Studies; Glomerulonephritis; Glomerulonephritis - diagnosis; Glomerulonephritis - epidemiology; Glomerulonephritis - mortality; Humans; Kidney; Kidney - pathology; Longitudinal Studies; Male; Middle Aged; Prognosis; Registries; Retrospective Studies; Survival; Survival Rate; Vasculitis
• ISSN: 0931-0509; 1460-2385
• DOI: 10.1093/ndt/gfs428

Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has a poor prognosis. In the current study, we assessed whether prognosis in these patients improved over the last three decades. In a large inception cohort, all consecutive patients with ANCA-associated glomerulonephritis were included between January 1979 and December 2009. Inclusion criteria were the presence of ANCA and the availability of a kidney biopsy. To assess renal and patient survival, patients were divided in three groups through time: 1979-89, 1990-2000 and 2001-09. A total of 181 patients were included. One-, 5- and 10-year survival was 77, 66 and 49%, respectively. Survival within the time groups was significantly different, yielding a hazard ratio for death of 2.9 for 1990-2000 and 3.9 for 1979-89 compared with 2001-09 (P < 0.001). Serum creatinine and active lesions as found in the kidney biopsy significantly decreased through the three decades. Both patient and renal survival in patients with ANCA-associated renal vasculitis have improved over the last three decades. We postulate that both earlier diagnosis and better therapeutic management of patients are responsible for this effect.