Association between age at disease onset of anti-neutrophil cytoplasmic antibody–associated vasculitis and clinical presentation and short-term outcomes

Abstract

Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Patients of 75 years and over with ANCA‐associated vasculitis have a lower relapse risk than younger patients: A multicentre cohort study

“…There was no difference in other clinical manifestations, nor in FFS, between the two groups. Similar findings have been described in other studies [6,7]. The present cohort, both age groups included, also had characteristics similar to two large French (n = 162 patients, mean age 61) and American cohorts (n = 347, mean age 58) [24,26].…”

“…The peak incidence rate of AAV is estimated at 65-75 years, and remains elevated even after 75 years of age [2][3][4]. Older patients have more frequently a diagnosis of MPA characterized by more frequent anti-myeloperoxidase antibody (MPO-ANCA) positivity, kidney involvement, and less ear nose throat (ENT) involvement as compared with younger AAV patients [5][6][7]. Induction treatment of AAV is efficient among older patients and remission rates are estimated between 74% and 97% [5,8,9].…”

Impact of the COVID-19 Pandemic on Kidney Diseases Requiring Renal Biopsy: A Single Center Observational Study

“…This is in line with our previous report that during the lockdown period in March and April 2020, an incidence-shift with a COVID-19 gap of non-diagnosed AAV based on renal biopsy was followed by a postlockdown phase in subsequent months with a compensatory increased incidence rate ( Hakroush and Tampe, 2021 ). According to the literature, various kidney diseases are preceded by a prodromal phase characterized by constitutional symptoms ( Monti et al, 2020 ). Diagnostic delay at this stage is common and has been demonstrated to be associated with mortality and end-stage renal disease ( Koldingsnes and Nossent, 2002 ; Houben et al, 2017 ).…”

Challenge of diagnosing ANCA-associated vasculitis during COVID-19 pandemic: a missed ‘window of opportunity’

“…According to literature, AAV is preceded by a prodromal phase characterised by constitutional symptoms that pursue a waxing and waning course. 3 Diagnostic delay at this stage is common, and it has been demonstrated that a longer prodromal phase associated with mortality 4 5 and end-stage renal disease. 5 AAV should therefore always be considered as a possible differential diagnosis in patients with constitutional symptoms without a clear explanation.…”