Journal
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
Volume 24, Issue 12, Pages 1460-1466Publisher
WILEY
DOI: 10.1111/1756-185X.14213
Keywords
anti-neutrophil cytoplasmic antibodies; fibrotic lung disease; interstitial lung disease; myeloperoxidase antibody; vasculitis
Categories
Ask authors/readers for more resources
While MPO-ANCA-positive ILD may appear clinically and radiologically similar to MPO-ANCA-negative ILD, the underlying pathophysiology is likely different and requires different treatment strategies, including monitoring for the development of ANCA-associated vasculitis and considering new treatment options.
Myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) are often detected in association with a variety of lung pathologies, the most common being interstitial lung disease (ILD). A growing cohort of patients are being diagnosed with MPO-ANCA in the context of ILD without ANCA-associated vasculitis. Clinically and radiologically, there is little to differentiate this cohort from MPO-ANCA-negative ILD patients; however, the pathophysiology is likely different and different treatments are likely required. We present here a brief summary of the proposed pathophysiology of MPO-ANCA-positive ILD, and a more detailed review of the latest evidence on management, including monitoring for development of ANCA-associated vasculitis, immunosuppression, anti-fibrotics, and novel agents that have yet to be trialled in human experiments.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available