TY - JOUR
T1 - The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With ANCA-Associated Vasculitis
AU - Bloom, Jessica L.
AU - Pickett-Nairn, Kaci
AU - Silveira, Lori
AU - Fuhlbrigge, Robert C.
AU - Cuthbertson, David
AU - Akuthota, Praveen
AU - Corbridge, Thomas C.
AU - Khalidi, Nader A.
AU - Koening, Curry L.
AU - Langford, Carol A.
AU - McAlear, Carol A.
AU - Monach, Paul A.
AU - Moreland, Larry W.
AU - Pagnoux, Christian
AU - Rhee, Rennie L.
AU - Seo, Philip
AU - Silver, Jared
AU - Specks, Ulrich
AU - Warrington, Kenneth J.
AU - Wechsler, Michael E.
AU - Merkel, Peter A.
N1 - Publisher Copyright:
© 2023 American College of Rheumatology.
PY - 2023/12
Y1 - 2023/12
N2 - Objective: This study examined the relationship between age at diagnosis and disease characteristics and damage in patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). Methods: Analysis 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 (41–65), 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. Results: Analysis 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 proteinase 3-ANCA positive. Children with GPA/MPA experienced more subglottic stenosis and alveolar hemorrhage; children and 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), whereas all other scores were not significantly different. 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. (Figure presented.).
AB - Objective: This study examined the relationship between age at diagnosis and disease characteristics and damage in patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). Methods: Analysis 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 (41–65), 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. Results: Analysis 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 proteinase 3-ANCA positive. Children with GPA/MPA experienced more subglottic stenosis and alveolar hemorrhage; children and 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), whereas all other scores were not significantly different. 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. (Figure presented.).
UR - http://www.scopus.com/inward/record.url?scp=85173429015&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85173429015&partnerID=8YFLogxK
U2 - 10.1002/art.42651
DO - 10.1002/art.42651
M3 - Article
C2 - 37433067
AN - SCOPUS:85173429015
SN - 2326-5191
VL - 75
SP - 2216
EP - 2227
JO - Arthritis and Rheumatology
JF - Arthritis and Rheumatology
IS - 12
ER -