Neutrophil membrane expression of proteinase 3 (PR3) is related to relapse in PR3-ANCA-associated vasculitis

AA Rarok, CA Stegeman, PC Limburg… - Journal of the …, 2002 - journals.lww.com
Wegener granulomatosis (WG) is strongly associated with the presence of antineutrophil
cytoplasm autoantibodies (ANCA) with specificity for proteinase 3 (PR3). Relapses of WG
are frequently preceded by a rise of autoantibody titer and PR3-ANCA are able to activate
primed neutrophils in vitro. Except being stored intracellularly and translocated to the cell
surface upon neutrophil stimulation, PR3 can also be detected on the surface of non-
stimulated neutrophils (membrane PR3 or mPR3), with an interindividual variability in …