2021
DOI: 10.1007/s10067-021-05790-9
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Mechanisms of thrombosis in ANCA-associated vasculitis

Abstract: Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have a two- to threefold greater risk of developing venous as well as arterial thrombotic events. Although such thrombotic events are more commonly seen during phases of active AAV, they are also recognized to occur during AAV in remission. Endothelial injury is a key pathogenic event in AAV. Endothelial injury can be caused by neutrophil activation and release of thrombogenic tissue factor into the circulation. Neutrophil ac… Show more

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Cited by 33 publications
(26 citation statements)
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“…This study indicated that the BVAS, especially the cutaneous, cardiovascular, and nervous system evaluation items, of anti-MYL6 antibody-positive MPA patients was lower than anti-MYL6 antibody-negative MPA patients and that the proportion of patients with remission 6 months after initiation of remission-induction therapy in anti-MYL6 antibody-positive MPA patients was signi cantly higher than in anti-MYL6 antibody-negative MPA patients. These ndings were consistent with reports of the involvement of NETs in cutaneous and nervous system lesions in AAV [33,34] and cardiovascular diseases [35]. Collective ndings suggested that anti-MYL6 antibody could be a disease-protective autoantibody.…”
Section: Discussionsupporting
confidence: 91%
“…This study indicated that the BVAS, especially the cutaneous, cardiovascular, and nervous system evaluation items, of anti-MYL6 antibody-positive MPA patients was lower than anti-MYL6 antibody-negative MPA patients and that the proportion of patients with remission 6 months after initiation of remission-induction therapy in anti-MYL6 antibody-positive MPA patients was signi cantly higher than in anti-MYL6 antibody-negative MPA patients. These ndings were consistent with reports of the involvement of NETs in cutaneous and nervous system lesions in AAV [33,34] and cardiovascular diseases [35]. Collective ndings suggested that anti-MYL6 antibody could be a disease-protective autoantibody.…”
Section: Discussionsupporting
confidence: 91%
“…Studies reporting PROMs in TAK (both adult-onset [ 18 21 ] and childhood-onset [ 22 ] forms) reporting original data on any PROM were included. Due to the paucity of randomized controlled trials (RCTs) in TAK [ 23 ], both RCTs and observational studies (with or without control group) were included. Review articles, case reports, editorials and letters to editors were excluded.…”
Section: Methodsmentioning
confidence: 99%
“…Remarkably, patients with ANCA-associated autoimmune disease and tumor patients share an increased risk of thrombotic events [ 115 , 166 ]. AAV increases the risk of arterial thrombotic events (ATE) and venous thromboembolism (VTE, including deep vein thrombosis and pulmonary embolism) by at least 2–3-fold [ 166 , 167 ]. Cancer increases the risk of VTE 4- to 7-fold and of ATE approximately 2-fold in the short term [ 168 , 169 , 170 ].…”
Section: “Impact Beyond Shelf Life”: Nets and Neutrophil-derived Evs ...mentioning
confidence: 99%
“…Although ATE and VTE are classified into two etiologically separate groups, common risk factors for both are well-documented [ 171 , 172 ]. TF has been identified as an important mediator of ANCA-associated thrombosis, which is released packaged in EVs and in NET-bound form by ANCA-activated neutrophils [ 166 ]. Vascular injury caused by ANCA-activated neutrophils may lead to additional exposure of TF from adventitial cells that surround blood vessels.…”
Section: “Impact Beyond Shelf Life”: Nets and Neutrophil-derived Evs ...mentioning
confidence: 99%