ANCA-Associated Vasculitides—An Update

Johanna Kegel,Torsten Kirsch

doi:10.4236/health.2014.614209

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This review describes the clinical diagnosis and therapy as well as the patho-physiology of ANCA-associated vasculitides with a specific focus on the interplay of ANCAs with activated neutrophils and the deleterious pathophysiological consequences of neutrophil-endothelium interaction.

Highlights

Vasculitides are defined by the presence of inflammatory leukocytes in vessel walls with reactive damage to mural structures
The vasculitides have been categorized by the sizes and types of blood vessels most commonly affected leading to a distinction between large, medium sized- and small vessel vasculitis [3]
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) comprise granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome). These diseases are characterized by Pauci-immune necrotizing small-vessel vasculitis and glomerulonephritis, combined with granulomatous inflammation, in the airways, in GPA and EGPA [5]

Summary

Introduction

Vasculitides are defined by the presence of inflammatory leukocytes in vessel walls with reactive damage to mural structures. This leads to the loss of vessel wall integrity, compromise of the lumen with downstream tissue ischemia and necrosis. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) comprise granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome). These diseases are characterized by Pauci-immune necrotizing small-vessel vasculitis and glomerulonephritis, combined with granulomatous inflammation, in the airways, in GPA and EGPA [5]. With a greater incidence in men, and the mortality ratio is 2.6 compared to the general population with most deaths related to infection due to the immunosuppressive therapy [7] [8]

Classification of Granulomatosis with Polyangiitis

ACR Criteria

Pathophysiology of GPA

Characteristics of ANCAs

Endothelium-Neutrophil Interactions in GPA

10. What Triggers GPA and Its Relapse?