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Understanding the drivers of granulomatosis with polyangiitis

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Published on Oct 5, 2015

Granulomatosis with polyangiitis (GPA) is a form of autoimmune vasculitis that is associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) directed against proteinase 3 (PR3). In this episode, Véronique Witko-Sarsat, Nathalie Thieblemont, and Katherine Martin discuss their work, which shows that PR3 expression is enhanced on the surface of apoptotic neutrophils from GPA patients. PR3-expressing apoptotic neutrophils were found to promote pro-inflammatory responses and prevent the development of regulatory T cells. Moreover, the authors show that this immune dysregulation was also present in GPA patient. Together, the results of this study provide important insight into the pathogenesis of GPA.

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