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Innate immune cells in the pathogenesis of primary systemic vasculitis

  • Review Article - Pathogenesis Reviews
  • Published:
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Abstract

Innate immune system forms the first line of defense against foreign substances. Neutrophils, eosinophils, erythrocytes, platelets, monocytes, macrophages, dendritic cells, γδ T cells, natural killer and natural killer T cells comprise the innate immune system. Genetic polymorphisms influencing the activation of innate immune cells predispose to development of vasculitis and influence its severity. Abnormally activated innate immune cells cross-talk with other cells of the innate immune system, present antigens more efficiently and activate T and B lymphocytes and cause tissue destruction via cell-mediated cytotoxicity and release of pro-inflammatory cytokines. These secreted cytokines further recruit other cells to the sites of vascular injury. They are involved in both the initiation as well as the perpetuation of vasculitis. Evidences suggest reversal of aberrant activation of immune cells in response to therapy. Understanding the role of innate immune cells in vasculitis helps understand the potential of therapeutic modulation of their activation to treat vasculitis.

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Abbreviations

AAV:

ANCA-associated vasculitis

ADA:

Adenosine deaminase

ANCA:

Anti-neutrophil cytoplasmic antibodies

AxV:

Annexin V

BD:

Behcet’s disease

BVAS:

Birmingham Vasculitis Activity Score

CCL:

Chemokine ligand

CCR:

Chemokine receptor

CD:

Cluster of differentiation

CD40L:

CD 40 ligand

CECR:

Cat eye syndrome chromosome region

DAMP:

Disease-associated molecular pattern

DC:

Dendritic cells

DNA:

Deoxyribonucleic acid

ECP:

Eosinophil cationic protein

EGPA:

Eosinophilic granulomatosis with polyangiitis

ERK:

Extracellular signal-regulated kinase

FcγR:

Fc gamma receptor

GCA:

Giant cell arteritis

γδ T cells:

Gamma delta T cells

GPA:

Granulomatosis with polyangiitis

GWAS:

Genome-wide associated studies

HLA:

Human leukocyte antigen

HMGB:

High mobility group box

HSP:

Henoch–Schonlein purpura

IFN:

Interferon

Ig:

Immunoglobulin

IL:

Interleukin

IVIG:

Intravenous immunoglobulin

KD:

Kawasaki’s disease

LAMP:

Lysosomal associated membrane protein

LPS:

Lipopolysaccharide

MAP:

Mitogen-activated protein

MDC:

Myeloid dendritic cell

MPA:

Microscopic polyangiitis

MPO:

Myeloperoxidase

mRNA:

Microribonucleic acid

NET:

Neutrophil extracellular traps

NOD:

Non-obese diabetic

NK cell:

Natural killer cell

NKT cell:

Natural killer T cell

PDC:

Plasmacytoid dendritic cell

PI3K:

Phosphatidyl inositol-3 kinase

PMA:

Phorbol myristate acetate

PMP:

Platelet-derived microparticles

PR-3:

Proteinase-3

RAG:

Recombinase-associated gene

RAGE:

Receptor for advanced glycation end products

SCID:

Severe combined immunodeficiency

TA:

Takayasu’s arteritis

TCR:

T cell receptor

Th:

Helper T lymphocytes

TLR:

Toll-like receptor

Treg:

T regulatory cells

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Acknowledgments

The authors would like to acknowledge Dr Armen Yuri Gasparyan for his valuable inputs into the search strategy and improvement of the manuscript.

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  1. Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Rae Bareily Road, Lucknow, Uttar Pradesh, 226 014, India

    Durga Prasanna Misra & Vikas Agarwal

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Misra, D.P., Agarwal, V. Innate immune cells in the pathogenesis of primary systemic vasculitis. Rheumatol Int 36, 169–182 (2016). https://doi.org/10.1007/s00296-015-3367-1

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