Anti-proteinase 3 (anti-PR3) antibodies are a subtype of anti-neutrophil cytoplasmic antibodies (ANCAs), specifically directed against proteinase 3, a serine protease located in the azurophilic granules of neutrophils and monocytes. Anti-PR3 is most commonly associated with granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis (AAV). These autoantibodies are believed to play a pathogenic role by activating neutrophils, promoting endothelial damage, and contributing to inflammation in small- to medium-sized blood vessels. The presence of anti-PR3 is a key biomarker for diagnosing GPA and can be detected using immunoassays such as ELISA or indirect immunofluorescence.
In clinical settings, anti-PR3 serves not only as a diagnostic tool but also as a prognostic marker. High titers of anti-PR3 are typically correlated with disease activity and may help guide treatment decisions and monitor for relapse. In research contexts, anti-PR3 is studied for its role in the immunopathogenesis of vasculitis and as a potential therapeutic target. Ongoing studies are investigating the mechanisms by which PR3 and its corresponding autoantibodies contribute to immune dysregulation, aiming to develop more targeted immunotherapies for ANCA-associated diseases.
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