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The immunopathogenesis of vasculitis 

The immunopathogenesis of vasculitis
Chapter:
The immunopathogenesis of vasculitis
Author(s):

Andrew McClean

and C.O.S. Savage

DOI:
10.1093/med/9780199579655.003.0054
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date: 04 April 2020

1. AAV is now considered an autoimmune disease, with ANCA having a pathogenic role.

2. As an autoimmune disease, the development of AAV must involve some loss of tolerance. This may result from genetic factors, environmental factors such as silica exposure, or perhaps as a result of an infective antigen that mimics endogenous antigen.

3. The primary target for injury in AAV is the endothelial cell, probably as a result of injury by PR3, MPO or elastase.

4. Although ANCA exerts its effects via neutrophil activation, there is growing evidence that monocytes, lymphocytes and the alternate pathway of the complement system are all intimately involved in the immunopathogenesis of AAV.

5. Our improving understanding of the immunopathogenesis of AAV is opening up possible new avenues for therapy, such as targeting of intracellular signalling.

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