Vasculitis & polymyalgia rheumatica

The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With Antineutrophil Cytoplasmic Antibody (ANCA)‐Associated Vasculitis

Arthritis RheumatolPublished Prospective cohort study

Authors' conclusion

Age at diagnosis is associated with clinical characteristics in AAV. Although VDI and AVID scores increase with age at diagnosis, this is driven by non-disease-specific damage items.

Study context

Population
Patients with Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic GPA (EGPA) in the Vasculitis Clinical Research Consortium (2013–2021)1,020 GPA/MPA, 357 EGPA
Exposure
Age at diagnosis: children (<18) vs young adults (18-40) vs middle-aged adults (41-65) vs older adults (>65)
Outcome
Damage scores (VDI, ANCA Vasculitis Index of Damage (AVID), disease-specific and non-disease-specific damage scores) by age at diagnosis
Methodological detailslongitudinal, multicentre consortium cohort, adjusted for disease duration, medications, tobacco and ANCA
  • Longitudinal
  • Multicentre consortium cohort
  • Adjusted for disease duration, medications, tobacco and ANCA

Main finding

As reported in the abstract

Damage scores by age at diagnosis, GPA/MPA
All damage scores increased with age at diagnosis for GPA/MPA (adjusted)
P < 0.001
Disease-specific damage score did not differ with age at diagnosis (GPA/MPA)
P = 0.44

Observational data: associations, not effects.

Further findings

Damage scores by age at diagnosis, EGPA

Vasculitis Damage Index (VDI) scores increased with age at diagnosis
P < 0.009
All other scores not significantly different

Clinical characteristics by age at diagnosis

Female predominance in GPA/MPA decreased with age at diagnosis
AAV in childhood more often GPA and PR3-ANCA positive
Children with GPA/MPA: more subglottic stenosis and alveolar hemorrhage
Children and young adults with EGPA: more alveolar hemorrhage, need for intubation and gastrointestinal involvement
Older adults (GPA/MPA): more neurologic manifestations

Original publication

The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With ANCA‐Associated Vasculitis. Arthritis Rheumatol. Published 5 October 2023. doi:10.1002/art.42651

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