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