Complement in antineutrophil cytoplasmic antibody-associated vasculitis
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REVIEW ARTICLE
The Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012
Complement in antineutrophil cytoplasmic antibody-associated vasculitis Jun Yuan • Min Chen • Ming-Hui Zhao
Received: 3 August 2012 / Accepted: 25 September 2012 Ó Japanese Society of Nephrology 2012
Abstract Antineutrophil cytoplasmic antibody (ANCA)associated vasculitis is a group of autoimmune disorders. It was previously assumed that the complement system is not involved in the development of ANCA-associated vasculitis due to its ‘‘pauci-immune’’ feature in renal histology. However, increasing evidence indicates that activation of the complement system, especially via the alternative complement pathway, plays a crucial role in the pathogenesis of ANCA-associated vasculitis. In this brief review, we discuss the evidence, including in vivo, J. Yuan M. Chen (&) M.-H. Zhao Renal Division, Department of Medicine, Peking University First Hospital, 100034 Beijing, China e-mail: [email protected] J. Yuan M. Chen M.-H. Zhao Institute of Nephrology, Peking University, 100034 Beijing, China J. Yuan M. Chen M.-H. Zhao Key Laboratory of Renal Disease, Ministry of Health of China, 100034 Beijing, China J. Yuan M. Chen M.-H. Zhao Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, 100034 Beijing, China J. Yuan M. Chen M.-H. Zhao Peking-Tsinghua Center for Life Sciences, 100034 Beijing, China J. Yuan Hubei University of Traditional Chinese Medicine, Wuchang, 430061 Wuhan, China J. Yuan Hubei Hospital of Traditional Chinese Medicine, Wuchang, 430061 Wuhan, China
in vitro, and clinical studies, for complement system activation in ANCA-associated vasculitis. Keywords
ANCA Vasculitis Complement
Introduction Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of autoimmune disorders, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and Churg–Strauss syndrome (CSS) [1]. These diseases are characterized by necrotizing small-vessel vasculitis. ANCA are the serological hallmarks for the above-mentioned vasculitides. ANCA are predominantly IgG autoantibodies directed against neutrophil cytoplasmic constituents, in particular proteinase 3 (PR3) and myeloperoxidase (MPO). The pathogenesis of AAV has not been fully elucidated. ANCA and neutrophils play a central role [2, 3]. The histopathological hallmark of ANCA-associated glomerulonephritis is ‘‘pauci-immune’’ necrotizing crescentic glomerulonephritis (NCGN), characterized by little or no glomerular staining for immunoglobulins and complements on renal histology by immunofluorescence microscopy examination. Therefore, it was previously assumed that the complement system is not involved in the pathogenesis of AAV. However, increasing studies suggest that complement plays a crucial role in the development of AAV. The complement system can be activated through the classical, alternative, or mannose-binding lectin (MBL) pathway [4]. The classical pathway is activated by immune complex. Th
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