Abstract:
:ANCA associated vasculitis is a serious, very often recurrent disease that despite the current standard treatment with high-dose glucocorticoids and either cyclophosphamide or rituximab, patients have a nine-fold increased mortality risk in the first year compared with healthy controls, attributed to infections, vasculitis activity, and renal disease. During the last few years, novel findings have suggested that activation of the complement system, in particular the alternative complement system, has a significant role in ANCA associated vasculitis pathogenesis. Detection of several components of this system in the circulation and urine reflects disease activity, and thus may be useful for clinical prognosis and to set up personalised treatments. In fact, some components of the complement system, such as C5a, might be potential targets for therapy. In this Review an update on clinical evidence for the role of complement activation in AAV is provided and subsequently we discuss potential therapeutic strategies that target complement components and open the way for clinical use of this target therapy in the near future.
journal_name
Mol Immunoljournal_title
Molecular immunologyauthors
Quintana LF,Kronbichler A,Blasco M,Zhao MH,Jayne Ddoi
10.1016/j.molimm.2019.06.018subject
Has Abstractpub_date
2019-08-01 00:00:00pages
394-398eissn
0161-5890issn
1872-9142pii
S0161-5890(19)30287-1journal_volume
112pub_type
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