Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease. / Atiskova, Yevgeniya; Wildner, Jan; Spitzer, Martin Stephan; Aries, Charlotte; Muschol, Nicole; Dulz, Simon.
in: ORPHANET J RARE DIS, Jahrgang 16, Nr. 1, 485, 20.11.2021.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
AU - Atiskova, Yevgeniya
AU - Wildner, Jan
AU - Spitzer, Martin Stephan
AU - Aries, Charlotte
AU - Muschol, Nicole
AU - Dulz, Simon
N1 - © 2021. The Author(s).
PY - 2021/11/20
Y1 - 2021/11/20
N2 - PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters.METHODS: Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3.RESULTS: Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = - 0.5; p < 0.01), cardiovascular MSSI (r = - 0.5; p < 0.01), lyso-Gb3 (r = - 0.6; p < 0.01)].CONCLUSION: We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker.
AB - PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters.METHODS: Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3.RESULTS: Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = - 0.5; p < 0.01), cardiovascular MSSI (r = - 0.5; p < 0.01), lyso-Gb3 (r = - 0.6; p < 0.01)].CONCLUSION: We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker.
U2 - 10.1186/s13023-021-02080-0
DO - 10.1186/s13023-021-02080-0
M3 - SCORING: Journal article
C2 - 34801073
VL - 16
JO - ORPHANET J RARE DIS
JF - ORPHANET J RARE DIS
SN - 1750-1172
IS - 1
M1 - 485
ER -