The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With ANCA‐Associated Vasculitis

Abstract

ObjectiveThis study examined the relationship between age at diagnosis and disease characteristics and damage in patients with ANCA‐associated vasculitis (AAV).MethodsAnalysis of a prospective longitudinal cohort of patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA) in the Vasculitis Clinical Research Consortium (2013‐2021). Disease cohorts were divided by age at diagnosis (years): children (< 18), young adults (18‐40), middle‐aged adults (> 40‐65), and older adults (> 65). Data included demographics, ANCA type, clinical characteristics, Vasculitis Damage Index (VDI) scores, ANCA Vasculitis Index of Damage (AVID) scores, and novel disease‐specific and non‐disease‐specific damage scores built from VDI and AVID items.ResultsAnalysis included data from 1020 patients with GPA/MPA and 357 with EGPA. Female predominance in GPA/MPA decreased with age at diagnosis. AAV in childhood was more often GPA and PR3‐ANCA positive. Children with GPA/MPA experienced more subglottic stenosis and alveolar hemorrhage, Children/young adults with EGPA experienced more alveolar hemorrhage, need for intubation, and gastrointestinal involvement. Older adults (GPA/MPA) had more neurologic manifestations. After adjusting for disease duration, medications, tobacco, and ANCA, all damage scores increased with age at diagnosis for GPA/MPA (p < 0.001) except the disease‐specific damage score which did not differ (p = 0.44). For EGPA, VDI scores increased with age at diagnosis (p < 0.009) while all other scores were not significantly different.ConclusionAge at diagnosis is associated with clinical characteristics in AAV. While VDI and AVID scores increase with age at diagnosis, this is driven by non‐disease‐specific damage items.image

The HLA region in ANCA-associated vasculitis: characterisation of genetic associations in a Scandinavian patient population

“…In our study, the HLA alleles with strongest suggestive association with organ involvements mirror the prevalence of these organ involvements in PR3-AAV and MPO-AAV, respectively. For instance, ENT involvement, occurring in ~89% of patients with PR3-AAV and ~52% of patients with MPO-AAV, 51 showed suggestive association with the HLA-DPB1 locus, implying that the signal of association is linked with the category PR3-ANCA positive AAV rather than the organ involvement itself. Preferably, for a complete dissection of genetic associations with organ involvements in AAV, analyses would be performed in PR3-AAV and MPO-AAV separately.…”