Related Experiment Video
Updated: May 12, 2026
An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
Damage accrual and predictors of mortality in ANCA-associated vasculitis: a retrospective observational study
Murat Bektaş1,2, Burak Ince3, Sibel Zaralı4
1Department of Internal Medicine, Division of Rheumatology Istanbul Faculty of Medicine, Istanbul, Türkiye. bektas.murat1988@gmail.com.
Insights
This study on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) found most patients develop organ damage. While damage scores decreased over time, mortality rates remained unchanged, highlighting persistent challenges in AAV management.
Area of Science:
- Rheumatology
- Internal Medicine
- Immunology
Background:
- Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) encompasses severe systemic autoimmune diseases.
- Long-term outcomes, including organ damage and mortality, require continuous evaluation in AAV patient cohorts.
Purpose of the Study:
- To investigate factors influencing damage and mortality in patients with AAV.
- To compare outcomes between different serotypes of AAV (c-ANCA/PR3+ vs. p-ANCA/MPO+).
Main Methods:
- Retrospective analysis of 254 AAV patients meeting Chapel Hill Consensus Conference (CHCC) criteria.
- Patients stratified by ANCA serotype (c-ANCA/PR3+ and p-ANCA/MPO+).
- Comparison of relapse, damage (Vasculitis Damage Index - VDI), and mortality data.
Main Results:
- 89.7% of patients developed organ damage; median VDI was 2.
- VDI scores decreased over time (1997-2011 vs. 2011-2021) and were higher in GPA than MPA.
- Five-year survival was 88.1%, overall survival 80.3%. Malignancy, severe infection, and persistent disease increased mortality risk.
Conclusions:
- The majority of AAV patients experience permanent organ damage.
- Despite a reduction in damage scores over time, overall mortality in AAV remains a significant concern.
- Factors like malignancy and infection critically impact AAV patient survival.
Abstract:
In this study, we aimed to evaluate the factors affecting the development of damage and mortality in patients with AAV treated at our tertiary referral center. This retrospective study included data on patients with AAV who fulfilled the Chapel Hill Consensus Conference (CHCC) criteria. Patients were divided into c-ANCA/PR3( +) and p-ANCA/MPO ( +) groups based on ANCA immunofluorescence and/or ELISA results, and relapse, damage, and mortality data were compared across the groups. Data from 254 patients (n = 136, 53.5% female) were included in the analysis. Clinical diagnosis was GPA in 186 (73.2%) and MPA in 68 (26.8%) patients. During the follow-up, 217 of 242 (89.7%) patients developed damage, and the median VDI score of the cohort was 2 (IQR: 2). VDI scores were higher in the first period (1997-2011) than in the second period (2011-2021) in the entire cohort (p = 0.012) and in patients with GPA compared with MPA (p = 0.034). Five-year and overall survival rates were 88.1% and 80.3% in the entire cohort; 87.8% and 81% in c-ANCA/PR3 ( +); 86.2% and 76% in p-ANCA/MPO ( +) (Log-Rank: p = 0.35); 91% and 84.5% in GPA; 81% and 67.2% in MPA patients (Log-Rank: p < 0.001). Development of malignancy, severe infection, and active/persistent disease after the induction phase were associated with higher mortality in patients with AAV. In our AAV cohort, permanent organ damage was detected in the majority of the patients. Although the median VDI score decreased over time, mortality did not change.
More Related Videos
Related Concept Videos
01:30Coronary Artery Disease I: Introduction
01:27Atherosclerosis II: Clinical Manifestations and Diagnostic Tests
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
01:30Acute Kidney Injury IV: Diagnostic Studies and Prevention