ABSTRACT

Microscopic polyangiitis (MPA) is a multisystem autoimmune vasculitis, associated

with antineutrophil cytoplasm antibodies (ANCA), and characterized by pauci-immune

necrotizing vasculitis of small blood vessels. It is one of three small vessel vasculitidies

commonly associated with ANCA, the others being Wegener’s granulomatosis (WG)

and Churg-Strauss syndrome (CSS). However, despite the common association with

ANCA, these disease entities have significant differences in their clinical fea-

tures and possibly in their underlying pathophysiology. As such, data on pathological

mechanisms should be extrapolated from one disease to another with caution.