腎臓学と治療学ジャーナル

ANCA Associated Glomerulonephritis- An In-Depth Review

Abstract

Abeer Kaldas ,Irfan Warraich ,Sharma S Prabhakar *

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.