Isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition - PubMed
4 hours ago
- #ANCA-ILD
- #Interstitial lung disease
- #Pulmonary fibrosis
- Isolated ANCA-ILD primarily affects older adults (50-70 years), with a slight male predominance (57%).
- MPO-ANCA positivity is observed in ~70% of patients, while PR3-ANCA accounts for 10-15%.
- Fibrotic patterns dominate, with UIP/UIP-like changes in 45-65% and NSIP in 15-30% of cases.
- Treatment includes glucocorticoids (58%), immunosuppressive therapies (31%), and antifibrotic agents (~26%).
- Functional decline occurs in 30-50% of patients, and radiological progression in 30-45%.
- Acute exacerbations reported in 19-25%, with up to 40% meeting progressive pulmonary fibrosis criteria.
- Progression to systemic AAV occurs in 10-40% of cases, mainly toward microscopic polyangiitis (MPA).
- Survival rates are comparable to ANCA-negative idiopathic interstitial pneumonia and AAV-ILD.