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. 2020 May 1;59(5):1076-1083.
doi: 10.1093/rheumatology/kez388.

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

Dominic McGovern  1 , Sam P Williams  1 , Katrina Parsons  2 , Tariq E Farrah  1   3 , Peter J Gallacher  1   3 , Eve Miller-Hodges  1   3 , David C Kluth  1 , Robert W Hunter  1   3 , Neeraj Dhaun  1   3
Affiliations

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

Dominic McGovern et al. Rheumatology (Oxford). .

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.

Keywords: ANCA; cyclophosphamide; frailty; glucocorticoid; methylprednisolone; rituximab; vasculitis.

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Figures

<sc>Fig</sc>. 1
Fig. 1
Patient survival in whole cohort (with 95% CI) .
<sc>Fig</sc>. 2
Fig. 2
Hazard of death during follow-up period Hazard ratios (with 95% CI) were computed in a Cox proportional hazards model, adjusted for frailty, age, sex, ANCA status, eGFR and CRP. All predictor variables were assessed at presentation. RCFS, Rockwood Clinical Frailty Scale; eGFR, estimate glomerular filtration rate.
<sc>Fig</sc>. 3
Fig. 3
Disease outcomes stratified by ANCA serotype A) Time to death. B) Time to remission. C) Time to relapse.
See this image and copyright information in PMC

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