Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.

Hereditary transthyretin amyloidosis is an autosomal dominant form of amyloidosis caused by an abnormality in transthyretin, with various ocular manifestations. Among these, ocular amyloid angiopathy has attracted attention because of its direct link to visual impairment and its correlation with sys...

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Main Authors: Shinji Kakihara, Takao Hirano, Junya Kitahara, Yorishige Matsuda, Akira Imai, Teruyoshi Miyahara, Toshinori Murata
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2022-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0275180
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author Shinji Kakihara
Takao Hirano
Junya Kitahara
Yorishige Matsuda
Akira Imai
Teruyoshi Miyahara
Toshinori Murata
author_facet Shinji Kakihara
Takao Hirano
Junya Kitahara
Yorishige Matsuda
Akira Imai
Teruyoshi Miyahara
Toshinori Murata
author_sort Shinji Kakihara
collection DOAJ
description Hereditary transthyretin amyloidosis is an autosomal dominant form of amyloidosis caused by an abnormality in transthyretin, with various ocular manifestations. Among these, ocular amyloid angiopathy has attracted attention because of its direct link to visual impairment and its correlation with systemic severity. We hypothesized that optical coherence tomography angiographic parameters would be useful biomarkers of amyloidosis systemic severity and investigated their correlation with the systemic severity score. The primary outcome was the correlation between the systemic severity score and choriocapillaris flow deficit percentage. Secondary outcomes were the correlations between the systemic severity score and retinal optical coherence tomography angiographic parameters, including foveal avascular zone size and circularity and superficial/deep/total retinal perfusion and vessel densities. The choroidal and retinal vasculature was quantified in 36 eyes from 36 patients (age, 51.8±12.1 years; disease duration, 13.4±6.2 years). Ten eyes had a history of vitrectomy for vitreous opacity. Choriocapillaris flow deficit percentage was not significantly correlated with the systemic severity score (Spearman's rank correlation: r = 2.96×10-2, p = 0.863). Similarly, foveal avascular zone size and circularity, and superficial/deep/total retinal perfusion and vessel densities were not significantly correlated with the systemic severity score. These results may indicate that optical coherence tomography angiographic parameters are not sufficient to predict amyloidosis severity.
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spelling doaj.art-a5b84aa7891c4bddbbb0d2701ca365e62022-12-22T03:38:36ZengPublic Library of Science (PLoS)PLoS ONE1932-62032022-01-01179e027518010.1371/journal.pone.0275180Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.Shinji KakiharaTakao HiranoJunya KitaharaYorishige MatsudaAkira ImaiTeruyoshi MiyaharaToshinori MurataHereditary transthyretin amyloidosis is an autosomal dominant form of amyloidosis caused by an abnormality in transthyretin, with various ocular manifestations. Among these, ocular amyloid angiopathy has attracted attention because of its direct link to visual impairment and its correlation with systemic severity. We hypothesized that optical coherence tomography angiographic parameters would be useful biomarkers of amyloidosis systemic severity and investigated their correlation with the systemic severity score. The primary outcome was the correlation between the systemic severity score and choriocapillaris flow deficit percentage. Secondary outcomes were the correlations between the systemic severity score and retinal optical coherence tomography angiographic parameters, including foveal avascular zone size and circularity and superficial/deep/total retinal perfusion and vessel densities. The choroidal and retinal vasculature was quantified in 36 eyes from 36 patients (age, 51.8±12.1 years; disease duration, 13.4±6.2 years). Ten eyes had a history of vitrectomy for vitreous opacity. Choriocapillaris flow deficit percentage was not significantly correlated with the systemic severity score (Spearman's rank correlation: r = 2.96×10-2, p = 0.863). Similarly, foveal avascular zone size and circularity, and superficial/deep/total retinal perfusion and vessel densities were not significantly correlated with the systemic severity score. These results may indicate that optical coherence tomography angiographic parameters are not sufficient to predict amyloidosis severity.https://doi.org/10.1371/journal.pone.0275180
spellingShingle Shinji Kakihara
Takao Hirano
Junya Kitahara
Yorishige Matsuda
Akira Imai
Teruyoshi Miyahara
Toshinori Murata
Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
PLoS ONE
title Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
title_full Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
title_fullStr Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
title_full_unstemmed Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
title_short Application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis.
title_sort application of optical coherence tomography angiography to assess systemic severity in patients with hereditary transthyretin amyloidosis
url https://doi.org/10.1371/journal.pone.0275180
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