PMID- 33841406
OWN - NLM
STAT- MEDLINE
DCOM- 20210914
LR  - 20210914
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 12
DP  - 2021
TI  - Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Update: Genetic 
      Pathogenesis.
PG  - 624848
LID - 10.3389/fimmu.2021.624848 [doi]
LID - 624848
AB  - Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is 
      characterized by the inflammation of small and medium vessels and presence of 
      proteinase 3-ANCA or myeloperoxidase-ANCA in the circulation. AAV comprises three 
      clinical subtypes: granulomatosis with polyangiitis (GPA), microscopic 
      polyangiitis (MPA), and eosinophilic GPA (EGPA). Although the pathogenesis of AAV 
      is still unclear, genetic and environmental factors and the immune system are 
      thought to be involved. Genetic factors have been confirmed to play an important 
      role in AAV. Genome-wide association studies have identified numerous genetic 
      variants in MHC and non-MHC regions associated with AAV. The strongest evidence 
      of MHC association in AAV is human leukocyte antigen (HLA)-DP. A significant 
      association between AAV and genetic variations in non-MHC regions, such as 
      CTLA-4, FCGR2A, PTPN22, SERPINA1, and TLR9 has also been found. Moreover, 
      different clinical subtypes of AAV have distinct genetic backgrounds. GPA is 
      associated with HLA-DP1, MPA with HLA-DQ, and EGPA with HLA-DRB4. These findings 
      could help elucidate the etiology of AAV and develop new biomarkers for diagnosis 
      and targeted therapy. Herein, we briefly summarize the updates on the genetic 
      pathogenesis and biomarkers of AAV.
CI  - Copyright (c) 2021 Li, Huang, Cai, Yuan and Sheng.
FAU - Li, Weiran
AU  - Li W
AD  - Institute of Dermatology and Department of Dermatology, The First Affiliated 
      Hospital, Anhui Medical University, Hefei, China.
AD  - Key Laboratory of Dermatology, Anhui Medical University, Ministry of Education, 
      Hefei, China.
FAU - Huang, He
AU  - Huang H
AD  - Institute of Dermatology and Department of Dermatology, The First Affiliated 
      Hospital, Anhui Medical University, Hefei, China.
AD  - Key Laboratory of Dermatology, Anhui Medical University, Ministry of Education, 
      Hefei, China.
FAU - Cai, Minglong
AU  - Cai M
AD  - Institute of Dermatology and Department of Dermatology, The First Affiliated 
      Hospital, Anhui Medical University, Hefei, China.
AD  - Key Laboratory of Dermatology, Anhui Medical University, Ministry of Education, 
      Hefei, China.
FAU - Yuan, Tao
AU  - Yuan T
AD  - Institute of Dermatology and Department of Dermatology, The First Affiliated 
      Hospital, Anhui Medical University, Hefei, China.
AD  - Key Laboratory of Dermatology, Anhui Medical University, Ministry of Education, 
      Hefei, China.
FAU - Sheng, Yujun
AU  - Sheng Y
AD  - Institute of Dermatology and Department of Dermatology, The First Affiliated 
      Hospital, Anhui Medical University, Hefei, China.
AD  - Key Laboratory of Dermatology, Anhui Medical University, Ministry of Education, 
      Hefei, China.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20210326
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
RN  - 0 (Biomarkers)
SB  - IM
MH  - Anti-Neutrophil Cytoplasmic Antibody-Associated 
      Vasculitis/diagnosis/*genetics/immunology
MH  - Antibodies, Antineutrophil Cytoplasmic/*blood
MH  - Biomarkers/blood
MH  - Genetic Predisposition to Disease
MH  - *Genetic Variation
MH  - Genome-Wide Association Study
MH  - Humans
MH  - Phenotype
MH  - Risk Assessment
MH  - Risk Factors
PMC - PMC8032971
OTO - NOTNLM
OT  - antineutrophil cytoplasmic antibody
OT  - genetic
OT  - genome-wide association studies
OT  - variation
OT  - vasculitis
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2021/04/13 06:00
MHDA- 2021/09/15 06:00
PMCR- 2021/01/01
CRDT- 2021/04/12 06:17
PHST- 2020/11/01 00:00 [received]
PHST- 2021/03/11 00:00 [accepted]
PHST- 2021/04/12 06:17 [entrez]
PHST- 2021/04/13 06:00 [pubmed]
PHST- 2021/09/15 06:00 [medline]
PHST- 2021/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2021.624848 [doi]
PST - epublish
SO  - Front Immunol. 2021 Mar 26;12:624848. doi: 10.3389/fimmu.2021.624848. eCollection 
      2021.