Abstract
Anti-neutrophil cytoplasmic antibodies (ANCA) are IgG antibodies directed against antigenic constituents of neutrophils contained in the azurophilic granules and monocyte lysosomes. Systemic vasculitis with ANCA [ANCA-associated vasculitides (AAV)] is a subgroup of life-threatening inflammatory disorders affecting small- to medium-sized vessels; immunosuppressants and glucocorticoids represent the current therapeutic mainstay. Although these agents have improved patients' survival, 25% present with severe adverse events, and standard therapy does not sustain remission. Therefore, an unmet need for safer and more effective therapies has prompted interest in biological agents. Continuous advances in the knowledge of AAV pathogenesis are paving the way to new biologicals that are now awaiting testing.
MeSH terms
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Animals
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Antibodies, Antineutrophil Cytoplasmic / immunology*
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Antibodies, Antineutrophil Cytoplasmic / metabolism
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Antibodies, Monoclonal / therapeutic use
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Antibodies, Monoclonal, Murine-Derived
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Antibody-Dependent Cell Cytotoxicity*
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Antibody-Producing Cells / drug effects
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Antibody-Producing Cells / metabolism
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Autoimmunity
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Endothelial Cells / immunology
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Endothelial Cells / metabolism*
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Humans
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Inflammation Mediators / immunology
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Inflammation Mediators / metabolism
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Neutrophil Activation / immunology*
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Neutrophils / immunology*
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Neutrophils / metabolism
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Paracrine Communication / immunology
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Respiratory Burst / genetics
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Respiratory Burst / immunology
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Rituximab
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Signal Transduction / immunology
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Vasculitis / immunology*
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Vasculitis / physiopathology
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Vasculitis / therapy*
Substances
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Antibodies, Antineutrophil Cytoplasmic
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Murine-Derived
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Inflammation Mediators
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Rituximab