PMID- 32493021
OWN - NLM
STAT- MEDLINE
DCOM- 20200811
LR  - 20200811
IS  - 1210-7875 (Print)
IS  - 1210-7875 (Linking)
VI  - 56
IP  - 2
DP  - 2020 Spring
TI  - Pathophysiology of ANCA-associated vasculitis.
PG  - 65-67
AB  - ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting 
      small vessels in various organ systems, accompanied by the presence of ANCA 
      antibodies in serum. AAV represents a group of the most common systemic 
      vasculitis in adulthood, and based on clinical manifestations this disease entity 
      includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly 
      Wegeners granulomatosis), microscopic polyangiitis and eosinophilic 
      granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to 
      other autoimmune diseases, AAV develops in patients with a predisposing genetic 
      background who have been exposed to causative environmental factors, such as 
      infections. The mechanisms by which ANCA antibodies cause vasculitis involves 
      excessive neutrophil activation, that subsequently leads to release 
      pro-inflammatory cytokines, reactive oxygen species and lytic enzymes. In 
      addition, activated neutrophils induce the formation of neutrophil extracellular 
      traps in a process called NETosis. The released neutrophil antigens are exposed 
      to the immune system via antigen presenting cells, which further stimulates 
      antibody production and creates a vicious circle with tissue destruction. 
      Understanding the pathogenesis of AAV represents the key which provides not only 
      optimal diagnosis and treatment, but also gives the pathologist a tool for deeper 
      insight into the morphological features of disease progression, including the 
      various stages of development and healing.
FAU - Lenka, Bartonova
AU  - Lenka B
FAU - Zdenka, Hruskova
AU  - Zdenka H
FAU - Eva, Honsova
AU  - Eva H
LA  - eng
PT  - Journal Article
TT  - Patofyziologicke mechanismy rozvoje ANCA asociovanych vaskulitid.
PL  - Czech Republic
TA  - Cesk Patol
JT  - Ceskoslovenska patologie
JID - 0050734
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
SB  - IM
MH  - Adult
MH  - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology
MH  - Antibodies, Antineutrophil Cytoplasmic
MH  - *Churg-Strauss Syndrome
MH  - *Granulomatosis with Polyangiitis
MH  - Humans
MH  - *Microscopic Polyangiitis
OTO - NOTNLM
OT  - AAV
OT  - ANCA associated vasculitis
OT  - GPA
OT  - MPA
OT  - pathophysiology
EDAT- 2020/06/05 06:00
MHDA- 2020/08/12 06:00
CRDT- 2020/06/05 06:00
PHST- 2020/06/05 06:00 [entrez]
PHST- 2020/06/05 06:00 [pubmed]
PHST- 2020/08/12 06:00 [medline]
AID - 122427 [pii]
PST - ppublish
SO  - Cesk Patol. 2020 Spring;56(2):65-67.