Proteinase 3 Autoreactivity in Anti-Neutrophil Cytoplasmic Antibody-associated vasculitis-Immunological versus clinical features.

Abstract

ANCA-associated vasculitis (AAV) is a group of chronic inflammatory diseases of small- and medium-sized vessels, which are broadly subdivided based on organ manifestations and disease-specific autoantibodies. The so called anti-neutrophil cytoplasmic antibodies (ANCA) mostly target one of the enzymes, proteinase 3 (PR3) or myeloperoxidase (MPO). Accumulating genetic data demonstrates that these two autoantibodies discriminate two distinct disease entities, more so than the clinical subdivision which is mainly criteria-based. Treatment of AAV includes heavy immunosuppression and is guided by which organs that are involved. Generally, patients with PR3-ANCA display higher risk for disease relapse than patients with MPO-ANCA. In this review, we will focus on the autoimmune features of PR3+ AAV and our current understanding of its triggers and the potential translation into clinical practice.

• Authors: Ravi K Sharma, Björn Lövström, Iva Gunnarsson, Vivianne Malmström
• Journal: Scandinavian journal of immunology (Scand J Immunol) Vol. 92 Issue 5 Pg. e12958 (Nov 2020) ISSN: 1365-3083 [Electronic] England
• PMID: 32794199 (Publication Type: Journal Article, Review)
• Copyright: © 2020 The Authors. Scandinavian Journal of Immunology published by John Wiley & Sons Ltd on behalf of The Scandinavian Foundation for Immunology.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• HLA-DP beta-Chains
• HLA-DPB1 antigen
• Peroxidase
• Myeloblastin

Topics

• Animals
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (immunology, metabolism, pathology)
• Antibodies, Antineutrophil Cytoplasmic (immunology)
• HLA-DP beta-Chains (immunology, metabolism)
• Humans
• Inflammation (immunology, metabolism)
• Models, Immunological
• Myeloblastin (immunology, metabolism)
• Peroxidase (immunology, metabolism)
• T-Lymphocyte Subsets (immunology, metabolism)