Myeloperoxidase and associated lung disease : review of the latest developments

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.