MORTALITY is higher in those with ANCA-positive interstitial lung disease (ILD) compared to those with ANCA-negative ILD, according to new research.
To gain a deeper understanding of the prevalence of ANCA positivity in patients with ILD from China, and the associations between ANCA positivity and survival and clinical characteristics, Tingting Wu, The First Affiliated Hospital of Ningbo University, Zhejiang, China, and colleagues, retrospectively reviewed data from 706 patients with ILD who had documented ANCA test results between March 2010–October 2023.
ANCA positivity was identified in 22.4% of patients (n=158). Of these patients, 58.2% developed ANCA positivity 41.6±31.4 months after diagnosis, on average. Compared to those who were ANCA-negative, those with ANCA-positive ILD were older, had a higher proportion of rheumatoid factor positivity, and had increased levels of serum inflammatory markers.
Notably, all-cause mortality was significantly higher (p=0.033) in those with ANCA-positive ILD (33.5%) compared to those with ANCA-negative ILD (25.0%). Additionally, associations between acute exacerbations of ILD and combination glucocorticoid and immunosuppressant treatment, and survival were identified in those with ANCA-positive ILD. Where acute ILD exacerbation was independently associated with shorter survival (hazard ratio [HR]: 2.40; 95% CI: 1.37–4.22; p=0.002), combined glucocorticoid and immunosuppressant treatment was independently associated with longer survival (HR: 0.30; 95% CI: 0.16–0.57; p<0.001).
In terms of high-resolution CT imaging patterns, usual interstitial pneumonia was commonest (56.3%), and those with ANCA-positive ILD exhibited greater proportions of oddly shaped cysts (p<0.001) and honeycombing (p<0.001) than their ANCA-negative counterparts.
Together, these findings highlight the importance of ANCA testing in patients with ILD. Early identification via serum testing or chest CT imaging markers could help to pre-empt potential adverse events and help future management planning. Future research aims could seek to further understand the pathophysiological mechanisms underlying ANCA-positive ILD and develop targeted treatment strategies to improve patient outcomes.
Reference
Wu T et al. Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in interstitial lung disease: a retrospective cohort study. Rheumatology (Oxford). 2025;DOI:10.1093/rheumatology/keaf108.
