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Antineutrophil Cytoplasmic Antibodies (ANCA): Diagnostic Utility and Potential Role in the Pathogenesis of Vasculitis
Branko Malenica
; Division of Immunology, Clinical Institute of Laboratory Diagnosis, Zagreb University Hospital Center, Zagreb, Croatia
Marija Rudolf
; Division of Immunology, Clinical Institute of Laboratory Diagnosis, Zagreb University Hospital Center, Zagreb, Croatia
Ana Kozmar
; Division of Immunology, Clinical Institute of Laboratory Diagnosis, Zagreb University Hospital Center, Zagreb, Croatia
Abstract
Antineutrophil cytoplasmic antibodies (ANCA) are a heterogeneous group of circulating antibodies directed toward the cytoplasmic constituents of neutrophils and monocytes. ANCA have been de scribed in various diseases including idiopathic systemic vasculitides, connective tissue diseases,
inflammatory bowel diseases, autoimmune liver diseases, infectious diseases,
and some drugs. ANCA recognize different target antigens such as proteinase
3 (PR3-ANCA), myeloperoxidase (MPO-ANCA), cathepsin G, lactoferrin, bactericidal/permeability-increasing protein (BPI), and some others. However,
only PR3-ANCA and MPO-ANCA are closely associated with systemic vasculitides, in particular Wegener´s granulomatosis, microscopic polyangiitis and its
renal limited manifestation, and Churg-Strauss syndrome. Both in vitro and in
vivo experimental data strongly support a pathogenic role for ANCA in vasculitis and glomerulonephritis.
Keywords
ANCA; PR3-ANCA; MPO-ANCA; vasculitis; pathogenesis
Hrčak ID:
88556
URI
Publication date:
1.4.2004.
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