Long-term outcomes in elderly patients with ANCA-associated vasculitis

Abstract

Objective ANCA-associated vasculitis (AAV) is a small vessel vasculitis that commonly presents in the elderly. However, there are few long-term outcome data for these patients. Here, we assessed long-term outcomes in a single-centre cohort of elderly patients with AAV. Additionally, we tested whether a pre-morbid frailty score could aid prognosis. Methods Using a prospectively-compiled dataset, we investigated patients over the age of 65 who presented with AAV between 2005 and 2017 to a regional vasculitis centre. We used a Cox model to determine the factors associated with mortality. We also compared outcomes in pre-specified subgroups stratified by baseline frailty score, ANCA serotype and induction immunosuppression (with cyclophosphamide, rituximab or mycophenolate mofetil used as the main glucocorticoid-sparing agent). Results 83 patients were included in the study and were followed for a median of 1203 days. Median age was 74 years (range 65–92). Two- and five-year survival in the overall cohort were 83% (95% CI 75, 92%) and 75% (95% CI 65, 86%), respectively. The median cumulative dose of oral prednisolone was 2030 mg during the first three months. Only one patient received intravenous glucocorticoids. Age, frailty score and CRP at presentation were independently associated with mortality; all deaths occurred in patients aged over 75 at presentation. Patients treated with a cyclophosphamide-based induction regimen tended to be younger than those treated with rituximab or mycophenolate mofetil. Survival was better in the cyclophosphamide-treated group. Conclusion In the contemporary era, the overall prognosis of AAV in elderly patients is good. Baseline frailty associates with disease outcomes including mortality. A low-dose glucocorticoid regimen (avoiding intravenous methylprednisolone) can be used to treat AAV effectively in elderly patients.

Glucocorticoid-free treatment of severe ANCA-associated vasculitis

“…Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare, small-vessel vasculitis with a peak in incidence in the seventh and eighth decades [1]. Previous studies have shown that older age and poorer performance status are associated with worse outcomes likely as a consequence of the disease and its treatment [2,3]. Also, best practice for elderly patients is unclear, as they are underrepresented in randomized controlled trials of induction immunosuppression in AAV; mean participant ages in the CYCLOPS, RAVE, MYCYC and PEXIVAS trials were 57, 53, 60 and 63 years, respectively [4][5][6][7].…”

Special Considerations in Geriatric Populations

“…Frailty has also been described in scleroderma, potentially associated with disease severity (24,25). In elderly patients with antineutrophil cytoplasmic antibody–associated vasculitis, increased mortality is associated with age, elevated C‐reactive protein levels, and presence of frailty (26). In summary, frailty has been studied in multiple rheumatic diseases and has been found to be associated with severity of disease and adverse outcomes.…”

Frailty in inflammatory bowel diseases: an emerging concept

“… 38 , 61 A study of frailty in older patients with ANCA-associated vasculitis also found that frailty conferred an increased risk for longer hospitalizations and mortality, independent of age and co-morbidities. 62 However, many patients in these studies were treated with corticosteroids, which may certainly contribute to frailty. Better defining the relationship between frailty, medications used to treat chronic immune-mediated diseases, and disease activity itself will be important to further the study of frailty.…”