Pathogenesis of ANCA-Associated Vasculitis: New Possibilities for Intervention

Autor: Wayel H. Abdulahad, Peter Heeringa, Coen A. Stegeman, Cees G. M. Kallenberg
Přispěvatelé: Groningen Kidney Center (GKC), Translational Immunology Groningen (TRIGR)
Rok vydání: 2013
Předmět:
Pathology
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis
T-Lymphocytes
Complement Pathway
Alternative

Microscopic Polyangiitis
ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
Churg-Strauss Syndrome
T-CELL-ACTIVATION
Glomerulonephritis
immune system diseases
Proteinase 3
Medicine
skin and connective tissue diseases
antibodies to proteinase 3 (PR3-ANCA)
pathogenesis
HUMAN NEUTROPHILS
Staphylococcal Infections
Silicon Dioxide
POLYANGIITIS WEGENERS
Nephrology
Experimental pathology
CRESCENTIC GLOMERULONEPHRITIS
Microscopic polyangiitis
Granulomatosis with polyangiitis
Vasculitis
INTERACTIONS IN-VIVO
medicine.medical_specialty
Myeloblastin
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
SMALL-VESSEL VASCULITIS
WEGENERS-GRANULOMATOSIS
Antigen
Humans
Genetic Predisposition to Disease
cardiovascular diseases
Peroxidase
Anti-neutrophil cytoplasmic antibody
PROTEINASE-3 PR3
business.industry
microscopic polyangiitis (MPA)
Histocompatibility Antigens Class II
Autoantibody
antibodies to myeloperoxidase (MPO-ANCA)
medicine.disease
ENDOTHELIAL-CELLS
Polyarteritis Nodosa
respiratory tract diseases
Immunology
Gene-Environment Interaction
granulomatosis with polyangiitis (GPA)
business
Zdroj: American Journal of Kidney Diseases, 62(6), 1176-1187. W B SAUNDERS CO-ELSEVIER INC
ISSN: 0272-6386
DOI: 10.1053/j.ajkd.2013.05.009
Popis: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) comprise granulomatosis with polyangiitis (GPA), primarily associated with antibodies to proteinase 3 (PR3-ANCA); microscopic polyangiitis (MPA); and eosinophilic granulomatosis with polyangiitis (EGPA), both principally associated with antibodies to myeloperoxidase (MPO-ANCA). Genetic and environmental factors are involved in their etiopathogenesis, with a possible role for silica exposure in AAVs and Staphylococcus aureus infection in GPA. The distinct associations of PR3-ANCA and MPO-ANCA with different HLA class II antigens, which are stronger than those with the associated diseases, suggest a pathogenic role for those ANCAs and indicate that GPA and MPA are different diseases. Both in vitro and in vivo experimental data have shown that MPO-ANCA can induce necrotizing small-vessel vasculitis and glomerulonephritis. The additional role of the alternative pathway of complement activation has been demonstrated in human and experimental pathology. Also, T cells seem to be involved in lesion development, particularly in the kidney. Granuloma formation, as seen in PR3-ANCA-associated GPA, is not well explained by the presence of autoantibodies in experimental models. Here, T cells seem crucial. Recently obtained insights into the pathogenesis of AAVs have led to more targeted treatment of these life-threatening diseases. (C) 2013 by the National Kidney Foundation, Inc.
Databáze: OpenAIRE