Pathophysiological importance of antineutrophil antibodies in vasculitis

• Published in: Current opinion in hematology Vol. 18; no. 1; p. 25
• Main Authors: Basu, Neil, Erwig, Lars-Peter
• Format: Journal Article
• Language: English
• Published: United States 01.01.2011
• Subjects: Animals; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - immunology; Antibodies, Antineutrophil Cytoplasmic - immunology; Antibody Specificity - immunology; Complement Activation - immunology; Complement System Proteins - immunology; Humans; Lymphocyte Activation - immunology; Lysosomal-Associated Membrane Protein 2 - immunology; T-Lymphocyte Subsets - immunology; Vasculitis - immunology
• ISSN: 1531-7048
• DOI: 10.1097/MOH.0b013e328340dc2a

We endeavour to provide a brief overview of the recent advances in understanding of how antineutrophil cytoplasmic antibodies (ANCAs) contribute to the pathophysiology of vasculitis. Substantial progress has been made in our understanding of the immunopathogenesis of ANCA-associated vasculitides. Compelling evidence from in-vitro studies and experimental models in conjunction with clinical trials has confirmed that ANCAs directly contribute to the evolution and progression of the disease process. A new ANCA, directed against human lysosome membrane protein-2 (LAMP-2), has recently been described as a sensitive and specific marker for renal vasculitis and we discuss its potential impact for diagnosis and therapy. Furthermore, high-throughput approaches are starting to identify genetic patterns that may identify patients likely to respond to specific therapy or having a high probability of relapse. It has become increasingly clear over the last two decades that ANCA IgG is pathogenic in vasculitis. Novel therapies aimed at selected cell populations or blocking specific pathogenic pathways offer hope for more selectively treating this heterogeneous group of patients, while avoiding nonspecific immunosuppression and its adverse effects.