Immunopathogenesis of ANCA-Associated Vasculitis

• Published in: International journal of molecular sciences 2020-10, Vol.21 (19), p.7319-27
• Main Authors: Kronbichler, Andreas, Lee, Keum Hwa, Denicolò, Sara, Choi, Daeun, Lee, Hyojeong, Ahn, Donghyun, Kim, Kang Hyun, Lee, Ji Han, Kim, HyungTae, Hwang, Minha, Jung, Sun Wook, Lee, Changjun, Lee, Hojune, Sung, Haejune, Lee, Dongkyu, Hwang, Jaehyuk, Kim, Sohee, Hwang, Injae, Kim, Do Young, Kim, Hyung Jun, Cho, Geonjae, Cho, Yunryoung, Kim, Dongil, Choi, Minje, Park, Junhye, Park, Junseong, Tizaoui, Kalthoum, Li, Han, Smith, Lee, Koyanagi, Ai, Jacob, Louis, Gauckler, Philipp, Shin, Jae Il
• Format: Article
• Language: English
• Subjects: ANCA; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - blood; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - classification; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - genetics; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - pathology; Antibodies, Antineutrophil Cytoplasmic - blood; Antibodies, Antineutrophil Cytoplasmic - genetics; Antigens; Antineutrophil cytoplasmic antibodies; biomarker; Biomarkers; Biomarkers - blood; Churg-Strauss Syndrome - blood; Churg-Strauss Syndrome - genetics; Churg-Strauss Syndrome - pathology; Dendritic cells; Disease; DNA methylation; Enzymes; Epigenetics; Granulomatosis; Granulomatosis with Polyangiitis - blood; Granulomatosis with Polyangiitis - genetics; Granulomatosis with Polyangiitis - pathology; Humans; Immunopathogenesis; Infections; Kidneys; Kinases; Leukocytes (eosinophilic); Leukocytes (neutrophilic); Life Sciences; Lymphocytes; Microscopic Polyangiitis - blood; Microscopic Polyangiitis - genetics; Microscopic Polyangiitis - pathology; Myeloblastin - genetics; Neutrophils; Pathogenesis; Peroxidase; Peroxidase - genetics; phenotype; Phenotypes; Physiological aspects; Precision medicine; Prognosis; Proteinase; Proteinase 3; Review; Serogroup; treatment; Tumor necrosis factor-TNF; Vasculitis
• ISSN: 1422-0067; 1661-6596; 1422-0067
• DOI: 10.3390/ijms21197319

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.