Eosinophilic granulomatosis with polyangiitis: etiopathogenesis, classification and clinical phenotypes

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease that can be classified as both hypereosinophilic condition and an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by granulomatous inflammation. The pathogenesis of EGPA not completely understood. It likely this Th2-mediated, blood tissue eosinophilia serves the main diagnostic criterion. hallmarks effectors organ damage in include asthma-associated necrotizing small-to-medium vessels eosinophilic proliferation. Endothelial injury vascular inflammation caused ANCA via activation circulating neutrophils. Two clinical phenotypes have been described based on detection ANCA: ANCA-negative manifestations hypereosinophilia (for example, pulmonary infiltrates cardiomyopathy) ANCA-positive signs glomerulonephritis, purpura, mononeuritis multiplex). Both were confirmed histological genomic research. However, these two coexisting mechanisms cannot separated practice. aim article to present current knowledge ANCA-mediated aspects pathogenesis, classification EGPA, consider prospects for future Conclusion. development dysfunction. This dysfunction means patients genetically determined predisposition recognize antigen HLA-DQ (human leukocyte DQ) alleles produce anti-myeloperoxidase autoantibodies later develop aberrant autoimmune process. Further comprehensive post-genomic studies are needed identify pathogenetic characterize molecular features phenotypes. elaboration endotypes will lead identification new activity biomarkers therapeutic targets improve diagnosis treatment outcomes.