ANCA-associated interstitial lung disease and interstitial pneumonia with autoimmune features

Skvortsov A.: Moscow State University, Moscow

Akulkina L.: Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia

Novikov P.: Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia

Bulanov N.: Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia

Moiseev S.: Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia

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Summary

Interstitial lung disease (ILD) is a recognized manifestation of ANCA-associated vasculitis. In addition, patients with idiopathic interstitial pneumonia and ANCA positivity but without other manifestations of systemic vasculitis (ANCA-ILD) have also been reported.

Aim

To evaluate the occurrence of ANCA-ILD among patients with interstitial pneumonia with autoimmune features (IPAF) entity.

Material and methods

In a retrospective cohort study, we recruited the consecutive patients with IPAF or ANCA-ILD. Data on clinical presentation, CT patterns, and autoimmunity laboratory tests results were collected.

Results

We studied 49 patients (37 females, median age 57.0 years) with IPAF and 4 patients (3 females, age 42 to 67 years) with ANCA-ILD. In the IPAF group, CT-patterns of lung disease included nonspecific interstitial pneumonia (NSIP) in 30 (61.2%) patients, usual interstitial pneumonia (UIP) in 7 (14.3%) patients, undifferentiated interstitial pneumonia in 6 (12.2%) patients, and organizing pneumonia (OP) in 6 (12.2%) patients. The median forced vital capacity (FVC) was 85.0% (62.9; 100.7), and the median DLCO was 56.0% (41.5;67.5). Two of 4 patients ANCA-ILD showed NSIP on CT, 1 patient had UIP, and 1 patients presented with undifferentiated interstitial pneumonia. Myeloperoxidase ANCA were found to be positive in 3 patients, and proteinase-3 ANCA in 1 patient. The median FVC was 58% (52.0; 63.0), and the median DLCO was 59% (47.0;60.5). Forty four (89,8%) of 49 patients with IPAF and all 4 patients with ANCA-ILD were treated with glucocorticoid and/or immunosuppressive agents, whereas 5 (10.2%) patients with IPAF were receiving nintedanib.

Conclusion

Our findings confirm that ANCA can be found in a proportion of patients with idiopathic interstitial pneumonia. Immunosuppressive therapy can be beneficial for patients with ANCA-ILD. In patients with progressive lung fibrosis, nintedanib should be considered.

Key words

ANCA, ILD, IPAF, interstitial lung disease.