Title:Saint John on Patmos: Revelations of the Role of Antineutrophil Cytoplasmic Antibody (ANCA) in Vasculitis
Volume: 27
Issue: 17
Author(s): Esther N. Pijnappel, Ger T. Rijkers and Frans J. van Overveld*
Affiliation:
- Department. of Science, University College Roosevelt, Middelburg,Netherlands
Keywords:
Antineutrophil cytoplasmic antibodies (ANCA), myeloperoxidase (MPO), neutrophils, polyangiitis,
proteinase-3 (PR3), immunofluorescence pattern (p-ANCA), vasculitis, antineutrophil cytoplasmic antibodies, cytoplasmic
immunofluorescence pattern (c-ANCA).
Abstract: Diagnosis of vasculitis is based on the presence of histologic features and serological testing
for antineutrophil cytoplasmic antibodies (ANCA). In patients with vasculitis, two types of ANCA have
been identified: ANCA directed against the neutrophil serine protease proteinase-3 (PR3) which results
in a cytoplasmic immunofluorescence pattern (c-ANCA) and ANCA directed against the neutrophil enzyme
myeloperoxidase (MPO), which results in a perinuclear immunofluorescence pattern (p-ANCA).
Question is if the presence of ANCA is the consequence of abnormal neutrophil adhesion, activation,
and apoptosis. Or is it, through mechanisms which are not totally clear for the moment, the cause of vasculitis.
In the latter case it has to be postulated that ANCA autoantigens are expressed on the cell surface
of viable, or activated, or early-apoptotic neutrophils.
