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

Background. 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. Methods. 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. Results. 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. Conclusions. 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.