Anti-neutrophil cytoplasmic antibodies (ANCAs), such as those directed towards proteinase 3 (PR3) and myeloperoxidase (MPO), are associated with a distinct form of small-vessel vasculitis, known as ANCA-associated vasculitis (AAV), a term that encompases granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The lesions mainly involve arterioles, veins and capillaries, and occasionally large arteries. The symptoms are reflected in multiple organs such as upper respiratory tract, lung and kidney [1].
Goodpasture’s or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients [2].
ANCA can be divided into cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA) and atypical ANCA (X-ANCA). The target antigen of c-ANCA is mainly protease 3 (PR3). In the pathology state, the protease-3 epitope binds to c-ANCA, mediates inflammation, and causes vascular damage [1].
The target antigen of p-ANCA is mainly MPO. Under pathological conditions, its conformational epitope located in the heavy chain region can bind to p-ANCA, mediate inflammation and cause vascular damage [1].
Under pathological conditions, the anti-GBM activates complement after binding to its own antigen, and binds to neutrophils and macrophages, resulting in the release of cytokines and lysozyme, destroying the structure of the basement membrane, resulting in local inflammation of related organs [2].
[1] Bossuyt X, Cohen Tervaert JW, Arimura Y, et al. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis[J]. Nat Rev Rheumatol. 2017 Nov;13(11):683-692.
[2] Henderson SR, Salama AD. Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease[J]. Nephrol Dial Transplant. 2018;33(2):196-202.
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