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Risk Factors for Pulmonary Involvement in Patients with ANCA-associated Vasculitis: A Systematic Review and
Ze Yang1,2, Yanzuo Wu1, Yanhao Chen1
1The First School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, 310053, China.
Clinical Reviews in Allergy & Immunology
|July 31, 2026
Summary
This study distinguishes pulmonary involvement (PI) phenotypes in ANCA-associated vasculitis (AAV), identifying distinct risk factors for interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH). Findings aid in personalized risk stratification and management of AAV-related lung conditions.
Area of Science:
- Rheumatology and Pulmonology
- Autoimmune Diseases
- Vasculitis Pathophysiology
Background:
- Pulmonary involvement (PI) in ANCA-associated vasculitis (AAV) significantly impacts patient outcomes.
- Existing research lacks a clear distinction between interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH) phenotypes within AAV.
- This gap hinders effective risk stratification and personalized treatment strategies for AAV patients with lung manifestations.
Purpose of the Study:
- To synthesize and differentiate the clinical patterns and risk factors associated with ILD and DAH in AAV.
- To provide a clinically actionable framework for improved risk stratification and management of PI in AAV.
Main Methods:
- A comprehensive meta-analysis of observational studies was conducted, searching seven databases up to April 1, 2026.
- Univariate and multivariate meta-analyses were employed to calculate pooled odds ratios (OR) and mean differences (MD).
- Nineteen studies involving 2,944 patients were included in the analysis.
Main Results:
- For ILD, independent risk factors included age >65 years (OR=3.60) and male gender (OR=2.57); MPO-ANCA positivity was more frequent (OR=2.29).
- DAH was associated with factors like proteinuria, high BVAS score, elevated creatinine and BUN, and PR3-ANCA positivity.
- Overall PI was independently predicted by age (OR=1.04 continuous, OR=4.20 >65 years), male gender (OR=3.08), and high BVAS (OR=3.03), with MPO-ANCA positivity being more frequent (OR=2.55).
Conclusions:
- This meta-analysis establishes distinct clinical profiles for ILD and DAH in AAV, differentiating them for the first time.
- ILD appears to be the primary driver of overall pulmonary involvement in AAV.
- The findings offer a valuable tool for clinicians to personalize the management and risk-stratify patients with AAV-related pulmonary conditions.
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