Rapidly progressive glomerulonephritis secondary to anti-GBM disease associated with MPO-ANCA: a case report

Abstract

Anti-glomerular basement membrane (GBM) disease, also known as "Goodpasture syndrome," is an autoimmune vasculitis that affects small vessels through the deposit of IgG antibodies against the glomerular and alveolar basal membranes which are visualized as linear deposits in the GBM. The target of these autoantibodies is the non-collagen-1 domain of type IV collagen on the GBM, which presents changes in the structure of the α-345 hexamer, leading to a conformational change that triggers an autoimmune response (Gulati and McAdoo 2018). ANCA-associated vasculitis (AAVs) is a necrotizing vasculitis with few-to-none immune deposits, also affecting small vessels, and is associated with specific antibodies for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA)