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

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abstract

  • 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.

authors

  • Monti, Sara
  • Craven, Anthea
  • Klersy, Catherine
  • Montecucco, Carlomaurizio
  • Caporali, Roberto
  • Watts, Richard
  • Merkel, Peter A
  • Luqmani, Raashid
  • Achilleos, Katerina
  • Adler, Matthew
  • Alba, Marco A
  • Alba, Marco A
  • Albert, Daniel A
  • Alibaz-Oner, Fatma
  • Allcoat, Paul
  • Amano, Koichi
  • Amarasuriya, Manishka
  • Amudala, Naomi A
  • Andrews, Jacqueline
  • Archer, Amy M
  • Arimura, Yoshihiro
  • Atukorala, Inoshi
  • Azevedo, Elsa
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  • Baslund, Bo
  • Basu, Neil
  • Baykal, Mahire
  • Berger, Christoph
  • Berglin, Ewa
  • Besada, Emilio
  • Bhardwaj, Mamta
  • Bischof, Antje
  • Blockmans, Daniel
  • Blood, Janet
  • Draibe, Juliana Bordignon
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publication date

  • February 1, 2021

has subject area