Diagnosis and treatment of ANCA-associated interstitial lung disease

In recent years, it was shown that patients with ANCA-associated vasculitis, particularly with microscopic polyangiitis with ANCA specific for myeloperoxidase (MPO-ANCA), can present with interstitial lung disease (ILD). In some cases, ILD precedes the development of ANCA-associated vasculitis or can be its main manifestation, affecting the long-term prognosis and determining the extent of immunosuppressive therapy. This article focuses on pathogenesis, diagnosis and treatment of ANCA-associated ILD, as well as direction of further investigations.

Key words

Anti-neutrophil cytoplasmic antibodies (ANCA), microscopic polyangiitis (MPA), interstitial lung disease (ILD).