Comparing outcomes of biopsy-proven anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis patients treated with cyclophosphamide in the 20th and 21st centuries: a 23-year study

Abstract

BackgroundAnti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a multisystem autoimmune disorder associated with significant morbidity and mortality. Approximately 80–90% of patients have circulating ANCAs. Long-term outcomes appear to be improving. This retrospective study analyses the incidence and patient outcomes over a period of 23 years at a single tertiary centre.MethodsOutcomes of patients diagnosed with AAV between 1 January 1988 and 31 December 2010 were collected retrospectively. Data including patient demographics, age of diagnosis, dates of starting renal replacement therapy, death and biochemistry results were collected. Patients were divided into two cohorts (1988–99 and 2000–10) and analysed using Stata software (StataCorp, College Station, TX, USA).ResultsA complete dataset was obtained for 273 patients. Of these patients, 101 were diagnosed between 1988 and 1999 while 172 were diagnosed between 2000 and 2010. The number of patients diagnosed with AAV increased from 2.2/million in 1988 to 10.3/million in 2010. A higher proportion of patients (56.4%) in the earlier cohort presented with creatinine >500 μmol/L compared with the later cohort (30.2%; P < 0.001). Overall patient survival improved significantly between the two cohorts. Cohort 1 had a median survival of 59 months compared with 125 months for Cohort 2 (P = 0.003).ConclusionsThis study shows that AAV is being diagnosed at an earlier stage, resulting in improved outcomes. This may be because of improvements in the management of AAV and chronic kidney disease.

The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies

“…A recently published study on renal AAV showed better renal survival in a cohort of patients diagnosed in 2000-2010 compared to an older cohort diagnosed in 1988-1999 and significantly lower creatinine levels at diagnosis in the recent cohort, supporting this assumption [258]. Similar findings with improved renal survival paralleled by better renal function at diagnosis, indicating shorter diagnostic delay, have been reported also in other studies in ANCA-associated glomerulonephritis [183,259].…”

Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study

“…Overall, the proportion of renal involvement was similar to that reported in the literature [ 12 ]; however, in earlier reports, up to one-third of those with antineutrophil cytoplasmic antibody–associated vasculitis with renal involvement went on to develop end-stage renal disease [ 16 ] which was higher than what was found in this study (granulomatosis with polyangiitis: 73/457, 16.0%; microscopic polyangiitis: 18/135, 13.3%). This could be related to the improvement of renal outcomes in granulomatosis with polyangiitis and microscopic polyangiitis patients over time reflecting the changes in diagnosis and management [ 16 , 17 ]. This could also reflect the fact that there was a possible bias as a result of less morbidity in participants from the online portal.…”