Vasculitis & polymyalgia rheumatica
Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A European Multicenter Study
Authors' conclusion
At baseline, higher fibrosis severity, UIP, and lower FVC% were associated with worse outcomes.
Study context
- Population
- Patients with AAV-ILD or ANCA-ILD, European multicentre (MPO-ANCA 85%)MPO-ANCA 85%
- Participants
- 162 patients
- Exposure
- AAV-ILD vs ANCA-ILD
- AAV-ILDn = 123
- ANCA-ILDn = 39
- Outcome
- FVC% decline, respiratory failure, and mortality
Methodological detailsmulticentre, European, central review of chest CT
- Multicentre
- European
- Central review of chest CT
Main finding
As reported in the abstract
Primary outcomes: annual FVC% decline by radiologic pattern, respiratory failure, mortality
- Annual FVC% change, usual interstitial pneumonia (UIP) pattern
- −1.99%
- Annual FVC% change, nonspecific interstitial pneumonia (NSIP) pattern (P = 0.35)
- −3.76%
- Annual FVC% change, other patterns
- +0.36%
- Respiratory failure
- 19%
- Respiratory failure predictor: fibrosis severity grade 4, HR (P = 0.029)
- 4.7
- Respiratory failure predictor: baseline FVC%, HR (P = 0.002)
- 0.95
- Died over median 4.2-year follow-up
- 48%
- Mortality predictor: age, HR (P = 0.04)
- 1.08
- Mortality predictor: baseline FVC%, HR (P = 0.05)
- 0.97
Observational data: associations, not effects.
Further findings
Mean FVC% change with rituximab (adjusted mixed-effects model) · 12 months
- Rituximab associated with mean FVC% improvement (P = 0.07)
- +6.02%
Baseline radiologic and clinical features
- UIP the most frequent radiologic pattern
- 57%
- Radiologic fibrosis grade >10%
- half
- Kidney involvement (most commonly Berden focal class)
- 73%
Radiologic progression
- Radiologic progression, mainly in younger patients with higher fibrosis severity grade
- ≈50%
Original publication
Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A European Multicenter Study. Arthritis Rheumatol. Published 13 March 2026. doi:10.
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