학술논문
The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With ANCA-Associated Vasculitis.
Document Type
article
Author
Corbridge, Thomas; Khalidi, Nader; Koening, Curry; Langford, Carol; McAlear, Carol; Monach, Paul; Moreland, Larry; Pagnoux, Christian; Rhee, Rennie; Seo, Philip; Silver, Jared; Specks, Ulrich; Warrington, Kenneth; Wechsler, Michael; Merkel, Peter; Bloom, Jessica; Pickett-Nairn, Kaci; Silveira, Lori; Fuhlbrigge, Robert; Cuthbertson, David; Akuthota, Praveen
Source
Arthritis and Rheumatology. 75(12)
Subject
Language
Abstract
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 (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