A proposed role of neutrophil extracellular traps and their interplay with fibroblasts in ANCA-associated vasculitis lung fibrosis.

Abstract

ANCA-associated vasculitides (AAV) comprise three diseases: granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. They are characterised by small vessel inflammation and have a broad range of clinical manifestations and multiorgan involvement which endanger the patient's life. An increasingly recognised complication of AAV, especially in MPA is lung fibrosis, for which no clearcut therapy in this context is available. The release of neutrophil extracellular traps (NETs) in these diseases has been related to the development of fibrosis, but the precise mechanisms are not fully unravelled. This review provides an overview of some of the important proteins known to compose NETs, and proposes some mechanisms by which these remarkable components may exert an impact on the different fibroblastic phenotypes leading to lung fibrosis.