Abstract
Inflammation of vasculature involving small to medium vessels associated with antineutrophil cytoplasmic antibodies or ANCA are collectively referred to as ANCA vasculitidis. In majority of such disorders there are few or no immune deposits and hence the term- “pauci immune vasculitis”. The three main conditions included in this group include microscopic polyangiitis, Granulomatosis with polyangiitis (GPA) - formerly known as Wegener’s granulomatosis, and Eosinophilic Granulomatosis with Polyangiitis (EGPA)-formerly known as Churg-Strauss Syndrome. There have been several advances in the last two decades in the classification, understanding of the pathogenesis and management of these conditions. To provide an update on the classification, pathology and pathogenesis and therapeutic advances for management of ANCA vasculitis is the focus of this in depth review.
Highlights
ANCA Associated Vasculitides (AAV) comprises a group of multi-systemic diseases affecting small-to-medium-sized vessels and is characterized by the presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) with specificity for either Proteinase-3 (PR3) or Myeloperoxidase (MPO)
We developed a more clear understanding of how ANCA exert their effects to cause disease
AAV is characterized by pauci-immunity and absence of immune complexes; compelling evidences suggest the role of complement
Summary
ANCA Associated Vasculitides (AAV) comprises a group of multi-systemic diseases affecting small-to-medium-sized vessels and is characterized by the presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) with specificity for either Proteinase-3 (PR3) or Myeloperoxidase (MPO). The kidneys are vascular organs and are targets for different types of systemic vasculitides, in particular those affecting small vessels. The primary renal sites for small-vessel vasculitides are the glomeruli the most common clinical presentations are those of glomerulonephritis. The characteristic kidney lesions in these conditions are pauci-immune focal and segmental necrotizing and crescentic glomerulonephritis (NCGN). Active pauci-immune small vessel vasculitis is typically associated with circulating ANCA antibodies (ANCA vasculitis). NCGN may occur without extra renal manifestations of disease. ANCA-associated-vasculitis (AAV) comprises three different diseases entities: 1- Eosinophilic Granulamatosis with Polyangiitis (EGPA) formerly known as Churg-Strauss Syndrome (CSS). 3- Granulomatosis with Polyangiitis (GPA) previously known as Wagener’s Granulomatosis (WG)
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