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

Sara Monti, Anthea Craven, Catherine Klersy, Carlomaurizio Montecucco, Roberto Caporali, Richard Watts, Peter A Merkel, Raashid Luqmani, DCVAS Collaborators, Bo Baslund (Member of study group)

18 Citations (Scopus)


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.

Original languageEnglish
JournalRheumatology (Oxford, England)
Issue number2
Pages (from-to)617-628
Number of pages12
Publication statusPublished - 1 Feb 2021


  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis
  • Antibodies, Antineutrophil Cytoplasmic/immunology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Morbidity/trends
  • Prognosis
  • Retrospective Studies
  • Risk Assessment/methods
  • Risk Factors
  • Survival Rate/trends
  • United Kingdom/epidemiology
  • Young Adult
  • anti-neutrophil cytoplasmic antibody-associated vasculitis
  • age
  • outcome


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