Trends in Long‐Term Outcomes Among Patients With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis With Renal Disease

Abstract

Objective How advances in the management of ANCA (anti-neutrophil cytoplasmic antibody)-associated vasculitis (AAV) have impacted long-term outcomes is still unclear. We examined temporal changes over 25 years in long-term clinical outcomes, including the impact of renal function at diagnosis (a potential marker of time to disease detection) and duration of cyclophosphamide use in AAV patients with renal involvement. Methods ANCA-positive, biopsy-proven patients with AAV diagnosed in 1985–2009 followed in the Glomerular Disease Collaborative Network inception cohort were included. Outcomes included the composite outcome of end-stage renal disease (ESRD) or death as well as relapse. Cox proportional hazard or competing risk regression models were adjusted for potential baseline confounders. Results Data from 544 patients were included in the analysis. There was a decreasing 5-year risk of ESRD or death over time (log rank test for trend: p < 0.001). After adjustment for baseline characteristics, the risk of relapse was similar across the time periods (test for trend: p = 0.45). Serum creatinine at baseline was the only significant predictor of an increased risk of ESRD or death (HR 1.11 per 1 mg/dL of serum creatinine [95% CI 1.04–1.18], p = 0.002). Conclusion In patients with renal disease secondary to AAV, over 25 years the risk of ESRD or death has decreased but the risk of relapse has not changed. A higher serum creatinine at diagnosis is associated with a higher risk of ESRD or death, suggesting that earlier disease detection is potentially an important measure to improve outcomes in AAV.

Impact of renal involvement on survival in ANCA-associated vasculitis

“…This is in line with the recent studies which have shown that the long-term outcomes of patients with ANCA-associated vasculitis have improved over the last decade reflecting the changes in diagnosis and management of these patients [15,16]. The other reason could be prolonged remission maintenance therapy along with low-dose corticosteroids for up to 5 years.…”

“…We had a higher proportion of patients with GPA in our study compared to MPA and renal-limited disease. This is in contrast to most studies from nephrology registries which have an equal or higher proportion of MPA and RLV [15,16]. Presenting creatinine level and age had a positive correlation with mortality in a study from UK [17].…”

Organisation of lymphocytic infiltrates in ANCA‐associated glomerulonephritis

“…Renal involvement in ANCA‐associated vasculitis causes necrotising crescentic glomerulonephritis often accompanied by considerable tubulointerstitial inflammation . Despite extensive treatment, a significant proportion of patients have resistant or recurrent disease activity and develop organ damage and ESRD . Markers for refractory disease activity are needed to identify patients at risk of perpetual inflammation to improve patient care and outcome.…”

Validation of the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire

“…Despite improvement in mortality and morbidity with newer treatment regimens, the risk of relapse in AAV is 35% over 5 years 3. Many patients experience persistent disease activity, long-term exposure to toxic therapies4 and the psychosocial impact of a serious illness 5…”