Proteinase-3 as an autoantigen and the development of granulomatosis with polyangiitis.

IF 2.1 4区医学 Q3 ALLERGY

Allergologia et immunopathologia Pub Date : 2025-09-01 eCollection Date: 2025-01-01 DOI:10.15586/aei.v53i5.1250

José Leonardo Balmaceda, Luis Rodríguez, Oscar Correa-Jiménez, Alberto Alfaro-Murillo, Emiro Buendía, Marlon Múnera

引用次数: 0

Abstract

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.

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蛋白酶-3作为自身抗原与肉芽肿合并多血管炎的发展。

人蛋白酶3 （hPR3）是丝氨酸蛋白酶型溶酶体酶。在自身免疫性血管炎中，hPR3的自身抗体似乎在抗中性粒细胞细胞质抗体（ANCA）相关血管炎（AAV）的发生中起作用，其中该蛋白是主要的自身抗原。事实上，抗hPR3抗体的患者有更严重的症状、复发和对免疫抑制治疗的抵抗，这支持了这种自身抗原在AAV的病理生理和严重程度中的重要作用。在这篇综述中，我们描述了hPR3在AAV的病理生理、诊断中的作用，以及对其治疗的一些看法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Allergologia et immunopathologia 医学-过敏

CiteScore

3.70

自引率

0.00%

发文量

131

审稿时长

6-12 weeks

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.