Emerging concepts in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis

@article{Flint2015EmergingCI,
  title={Emerging concepts in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis},
  author={Shaun M Flint and Eoin F. McKinney and Kenneth G. C. Smith},
  journal={Current Opinion in Rheumatology},
  year={2015},
  volume={27},
  pages={197–203}
}
Purpose of reviewAntineutrophil cytoplasmic antibodies (ANCAs) remain central to our current understanding of the pathogenesis of ANCA-associated vasculitis (AAV), and this review considers recent developments in the context of four key questions: are there targets for ANCA beyond myeloperoxidase (MPO) and proteinase 3 (PR3); are all ANCA pathogenic; how are ANCAs generated; and how do ANCA cause disease? Recent findingsB-cell epitope mapping raises the possibility that only a subset of ANCA… 

Immunopathogenesis of ANCA-Associated Vasculitis

TLDR
The current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers are summarized and it is illustrated how the extending knowledge of the Immunopathogenesis will likely translate into development of a personalized medicine approach in the management of AN CA-associated Vasculitis.

Pathogenesis and therapeutic interventions for ANCA-associated vasculitis

TLDR
Advances in the understanding of anti-neutrophil cytoplasmic antibody-associated vasculitis are described and promising new treatments that target B cells, T cells and cytokines are generated; potential novel approaches targeting additional cells or molecules are also discussed.

Presence of dual anti-MPO and anti-PR3 antibodies in Systemic Lupus Erythematosus/ANCA-Associated Vasculitis

TLDR
This is the first report of a case of SLE/AAV involving AAV (necrotising pauci-immune GN) with both anti-MPO and anti-PR3 antibodies, reported in the literature as antimyeloperoxidase antibodies (MPO) rather than antiproteinase 3 (PR3) antibodies.

Neutrophils from ANCA-associated vasculitis patients show an increased capacity to activate the complement system via the alternative pathway after ANCA stimulation

TLDR
This study shows that primed and ANCA-stimulated neutrophil from AAV patients have a greater ability to activate the alternative complement pathway compared to primed neutrophils from healthy controls, highlighting the therapeutic potential of C5a and other complement blockade.

Small Vessel Vasculitis - To ANCA and Beyond

TLDR
Understanding of autoantibodies in small vessel vasculitis is transformed and quantifying of ANCA and antibodies to MPO or PR3 show they reflect disease activity poorly and cannot be used to predict relapses, either in those treated with traditional immunosuppressive drugs or with anti-B cell therapy rituximab.

The Presence of Anti-Lactoferrin Antibodies in a Subgroup of Eosinophilic Granulomatosis with Polyangiitis Patients and Their Possible Contribution to Enhancement of Neutrophil Extracellular Trap Formation

TLDR
It is suggested that aLf enhance NET formation induced by PMA and are associated with disease activity of EGPA and the effect was abolished completely by absorption of the aLF.

Pathogenic Role of ANCA in Small Vessel Inflammation and Neutrophil Function

TLDR
A vicious circle emerges as NETs endowed with ANCA targets promote ANCA generation, and multiple factors induce the generation of ANCA, which in turn have the ability to promote neutrophil activation and progression towards neutrophils extracellular trap (NET) development.

Granulomatosis with polyangiitis: update and key concepts

TLDR
Key aspects of granulomatosis with polyangiitis are reviewed, including aspects such as epidemiology, pathophysiology, clinical manifestations, diagnosis and treatment, which are fundamental to the development of new therapies for disease management.

Renal Tissue miRNA Expression Profiles in ANCA-Associated Vasculitis—A Comparative Analysis

TLDR
A considerable subset of differentially expressed miRNAs was related to macrophage and lymphocyte polarization and cytokines previously deemed important in AAV pathogenesis, lending credence to the obtained results.

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