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Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms

BACKGROUND: Proliferative vasculopathy (PV) associated antiphospholipid syndrome (APS) in the central nervous system is a rare un(der)recognized form of extra-criteria manifestations of APS. This study investigated the angiographic characteristics of cerebral and cervical arteries in patients with P...

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Autores principales: Yeo, Jina, Hwang, Inpyeong, Sohn, Chul-Ho, Lee, Eunyoung Emily, Lee, Soon-Tae, Lee, Eun Bong, Park, Jin Kyun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9252304/
https://www.ncbi.nlm.nih.gov/pubmed/35795628
http://dx.doi.org/10.3389/fmed.2022.913203
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author Yeo, Jina
Hwang, Inpyeong
Sohn, Chul-Ho
Lee, Eunyoung Emily
Lee, Soon-Tae
Lee, Eun Bong
Park, Jin Kyun
author_facet Yeo, Jina
Hwang, Inpyeong
Sohn, Chul-Ho
Lee, Eunyoung Emily
Lee, Soon-Tae
Lee, Eun Bong
Park, Jin Kyun
author_sort Yeo, Jina
collection PubMed
description BACKGROUND: Proliferative vasculopathy (PV) associated antiphospholipid syndrome (APS) in the central nervous system is a rare un(der)recognized form of extra-criteria manifestations of APS. This study investigated the angiographic characteristics of cerebral and cervical arteries in patients with PV associated with antiphospholipid antibodies (aPLs). METHODS: Patients with aPLs, neurologic symptoms and diffuse luminal narrowing on brain or neck magnetic resonance angiography were selected from electronic medical records. Vascular wall and intraluminal pathology were examined by high-resolution vessel wall MR imaging (VW-MRI). RESULTS: A total of 11 patients (six men and five women) with PV-aPL, of median (interquartile range) age 42 (34–61) years, were included. Median anticardiolipin antibodies IgG titer was 78.9 (28.2–134.0) units and anti-beta 2 glycoprotein I antibodies (aB2GPIs) IgG titer was 227.2 (0.0–1012.1) units. All patients had diffuse luminal narrowing in the carotid basilar and/or cerebral arteries, five in the internal carotid artery (ICA); two each in the middle cerebral artery (MCA) and vertebral artery; and one each in the basilar artery (BA) and posterior cerebral artery. On VW-MRI, four patients showed concentric thickening of the vascular walls of the ICA and/or MCA and two showed mild eccentric wall thickening of the ICA or BA. All patients received antithrombotic treatment. In two patients with extremely high aB2GPIs titer, diffuse narrowing progressed despite treatment with antithrombotic agents on follow-up imaging. CONCLUSIONS: This study suggests that PV-aPL might be a distinct extra-criteria manifestation of APS that can manifest as long-segmental diffuse stenosis of cerebral and cervical arteries. It should be considered in relatively young patients with neurologic symptoms and aPLs.
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spelling pubmed-92523042022-07-05 Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms Yeo, Jina Hwang, Inpyeong Sohn, Chul-Ho Lee, Eunyoung Emily Lee, Soon-Tae Lee, Eun Bong Park, Jin Kyun Front Med (Lausanne) Medicine BACKGROUND: Proliferative vasculopathy (PV) associated antiphospholipid syndrome (APS) in the central nervous system is a rare un(der)recognized form of extra-criteria manifestations of APS. This study investigated the angiographic characteristics of cerebral and cervical arteries in patients with PV associated with antiphospholipid antibodies (aPLs). METHODS: Patients with aPLs, neurologic symptoms and diffuse luminal narrowing on brain or neck magnetic resonance angiography were selected from electronic medical records. Vascular wall and intraluminal pathology were examined by high-resolution vessel wall MR imaging (VW-MRI). RESULTS: A total of 11 patients (six men and five women) with PV-aPL, of median (interquartile range) age 42 (34–61) years, were included. Median anticardiolipin antibodies IgG titer was 78.9 (28.2–134.0) units and anti-beta 2 glycoprotein I antibodies (aB2GPIs) IgG titer was 227.2 (0.0–1012.1) units. All patients had diffuse luminal narrowing in the carotid basilar and/or cerebral arteries, five in the internal carotid artery (ICA); two each in the middle cerebral artery (MCA) and vertebral artery; and one each in the basilar artery (BA) and posterior cerebral artery. On VW-MRI, four patients showed concentric thickening of the vascular walls of the ICA and/or MCA and two showed mild eccentric wall thickening of the ICA or BA. All patients received antithrombotic treatment. In two patients with extremely high aB2GPIs titer, diffuse narrowing progressed despite treatment with antithrombotic agents on follow-up imaging. CONCLUSIONS: This study suggests that PV-aPL might be a distinct extra-criteria manifestation of APS that can manifest as long-segmental diffuse stenosis of cerebral and cervical arteries. It should be considered in relatively young patients with neurologic symptoms and aPLs. Frontiers Media S.A. 2022-06-20 /pmc/articles/PMC9252304/ /pubmed/35795628 http://dx.doi.org/10.3389/fmed.2022.913203 Text en Copyright © 2022 Yeo, Hwang, Sohn, Lee, Lee, Lee and Park. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Yeo, Jina
Hwang, Inpyeong
Sohn, Chul-Ho
Lee, Eunyoung Emily
Lee, Soon-Tae
Lee, Eun Bong
Park, Jin Kyun
Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title_full Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title_fullStr Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title_full_unstemmed Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title_short Proliferative Vasculopathy Associated With Antiphospholipid Antibodies in Patients With Neurological Symptoms
title_sort proliferative vasculopathy associated with antiphospholipid antibodies in patients with neurological symptoms
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9252304/
https://www.ncbi.nlm.nih.gov/pubmed/35795628
http://dx.doi.org/10.3389/fmed.2022.913203
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