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

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 (<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.

• Authors: Jessica L Bloom, Kaci Pickett-Nairn, Lori Silveira, Robert C Fuhlbrigge, David Cuthbertson, Praveen Akuthota, Thomas C Corbridge, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Paul A Monach, Larry W Moreland, Christian Pagnoux, Rennie L Rhee, Philip Seo, Jared Silver, Ulrich Specks, Kenneth J Warrington, Michael E Wechsler, Peter A Merkel, Vasculitis Clinical Research Consortium
• Journal: Arthritis & rheumatology (Hoboken, N.J.) (Arthritis Rheumatol) Vol. 75 Issue 12 Pg. 2216-2227 (12 2023) ISSN: 2326-5205 [Electronic] United States
• PMID: 37433067 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: © 2023 American College of Rheumatology.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic

Topics

• Child
• Middle Aged
• Young Adult
• Humans
• Female
• Aged
• Male
• Antibodies, Antineutrophil Cytoplasmic
• Prospective Studies
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Granulomatosis with Polyangiitis (complications, epidemiology, drug therapy)
• Microscopic Polyangiitis (complications, epidemiology)
• Hemorrhage
• Churg-Strauss Syndrome