Home > D. General pathology > Blood and immunity > Dysimmune diseases > granulomatosis with polyangiitis
granulomatosis with polyangiitis
MIM.608710
Friday 8 July 2005
Wegener granulomatosis; GPA
Definition: Wegener granulomatosis is a systemic disease with a complex genetic background. It is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient sera.
Images
mastoid
Wegener’s Granulomatosis in lung with vasculitis
ANCAs
These ANCAs are antibodies to a defined target antigen, proteinase-3 (PR3) (MIM.177020), that is present within primary azurophil granules of neutrophils (PMNs) and lysozymes of monocytes.
On cytokine priming of PMNs, PR3 translocates to the cell surface, where PR3-ANCAs can interact with their antigens and activate PMNs.
PMNs from patients with active WG express PR3 on their surface, produce respiratory burst, and release proteolytic enzymes after activation with PR3-ANCAs. The consequence is a self-sustaining inflammatory process. (14968360)
Localization
pulmonary Wegener granulomatosis
Predisposition
Associations
Wegener granulomatosis (8296646)
Henoch-Schönlein purpura (8296646)
References
Yi ES, Colby TV. Wegener’s granulomatosis. Semin Diagn Pathol. 2001 Feb;18(1):34-46. PMID: 11296992