José Leonardo Balmaceda, Luis Rodríguez, Oscar Correa-Jiménez, Alberto Alfaro-Murillo, Emiro Buendía, Marlon Múnera
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Proteinase-3 as an autoantigen and the development of granulomatosis with polyangiitis.
Human proteinase 3 (hPR3) is a lysosomal enzyme of the serine protease type. In autoimmune vasculitis, autoantibodies to hPR3 appear to have a role in the inception of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), where this protein is the main autoantigen. Indeed, patients with antibodies against hPR3 have more severe symptoms, relapses, and resistance to immunosuppressive therapies, supporting an important role for this autoantigen in the pathophysiology and severity of AAV. In this review, we describe what is known about the role of hPR3 in pathophysiology, diagnosis of AAV, and some perspectives on its treatment.
期刊介绍:
Founded in 1972 by Professor A. Oehling, Allergologia et Immunopathologia is a forum for those working in the field of pediatric asthma, allergy and immunology. Manuscripts related to clinical, epidemiological and experimental allergy and immunopathology related to childhood will be considered for publication. Allergologia et Immunopathologia is the official journal of the Spanish Society of Pediatric Allergy and Clinical Immunology (SEICAP) and also of the Latin American Society of Immunodeficiencies (LASID). It has and independent international Editorial Committee which submits received papers for peer-reviewing by international experts. The journal accepts original and review articles from all over the world, together with consensus statements from the aforementioned societies. Occasionally, the opinion of an expert on a burning topic is published in the "Point of View" section. Letters to the Editor on previously published papers are welcomed. Allergologia et Immunopathologia publishes 6 issues per year and is included in the major databases such as Pubmed, Scopus, Web of Knowledge, etc.