Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-<i>sodium-fluoride</i> (18F-NaF) in the skin and vessel walls with positron emission tomograp...

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Main Authors: Antonio Gutierrez-Cardo, Eugenia Lillo, Belén Murcia-Casas, Juan Luis Carrillo-Linares, Francisco García-Argüello, Purificación Sánchez-Sánchez, Alejandro Rodriguez-Morata, Isabel Baquero Aranda, Miguel Ángel Sánchez-Chaparro, María García-Fernández, Pedro Valdivielso
Format: Article
Language:English
Published: MDPI AG 2020-05-01
Series:Journal of Clinical Medicine
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Online Access:https://www.mdpi.com/2077-0383/9/5/1393
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author Antonio Gutierrez-Cardo
Eugenia Lillo
Belén Murcia-Casas
Juan Luis Carrillo-Linares
Francisco García-Argüello
Purificación Sánchez-Sánchez
Alejandro Rodriguez-Morata
Isabel Baquero Aranda
Miguel Ángel Sánchez-Chaparro
María García-Fernández
Pedro Valdivielso
author_facet Antonio Gutierrez-Cardo
Eugenia Lillo
Belén Murcia-Casas
Juan Luis Carrillo-Linares
Francisco García-Argüello
Purificación Sánchez-Sánchez
Alejandro Rodriguez-Morata
Isabel Baquero Aranda
Miguel Ángel Sánchez-Chaparro
María García-Fernández
Pedro Valdivielso
author_sort Antonio Gutierrez-Cardo
collection DOAJ
description Pseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-<i>sodium-fluoride</i> (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomography. This was an observational study including 18 patients with PXE. Vascular calcification was measured in Agatston units, and deposition in the skin and vessel walls was shown using target-to-background ratio (TBR). Severity of the disease was scored by Phenodex. We found higher vascular calcification in the popliteal, femoral, and aortic arch vessels compared to other vascular regions; however, the uptake of radiotracer was the highest in the aorta and femoral arteries. In the skin, the highest uptake was observed in the neck and the axillae. There was no significant association between 18F-NaF deposition in the arteries or skin and the global Phenodex score. In contrast, the Phenodex score was significantly associated in univariate analyses with the averaged vascular calcium score (<i>p</i> < 0.01). In the neck, patients with higher skin Phenodex scores exhibited higher radiotracer uptake. As a conclusion, because vascular calcification is physiological, our data suggested that the detection of cutaneous (neck) 18F-NaF deposits might serve to monitor the calcification process in the short-term for patients with PXE.
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spelling doaj.art-abb26b9731844f9495a19169fb47431a2023-11-19T23:50:05ZengMDPI AGJournal of Clinical Medicine2077-03832020-05-0195139310.3390/jcm9051393Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma ElasticumAntonio Gutierrez-Cardo0Eugenia Lillo1Belén Murcia-Casas2Juan Luis Carrillo-Linares3Francisco García-Argüello4Purificación Sánchez-Sánchez5Alejandro Rodriguez-Morata6Isabel Baquero Aranda7Miguel Ángel Sánchez-Chaparro8María García-Fernández9Pedro Valdivielso10Molecular Image Unit, Nuclear Medicine, Fundación General de la Universidad de Málaga, 29010 Málaga, SpainMolecular Image Unit, Nuclear Medicine, Fundación General de la Universidad de Málaga, 29010 Málaga, SpainInternal Medicine Unit, Hospital Universitario Virgen de la Victoria, 29010 Málaga, SpainInternal Medicine Unit, Hospital Universitario Virgen de la Victoria, 29010 Málaga, SpainMolecular Image Unit, Radiopharmacy, Fundación General de la Universidad de Málaga, 29010 Málaga, SpainDepartment of Medicine and Dermatology, University of Málaga, Instituto de Investigación Biomédica de Málaga (IBIMA), 29010 Málaga, SpainUnit of Angiology and Vascular Surgery, Hospital Quirón, 29004 Málaga, SpainUnit of Ophtalmology, Hospital Universitario Virgen de la Victoria, 29010 Málaga, SpainDepartment of Medicine and Dermatology, University of Málaga, Instituto de Investigación Biomédica de Málaga (IBIMA), 29010 Málaga, SpainDepartment of Human Physiology, University of Málaga, and Instituto de Investigación Biomédica de Málaga (IBIMA), 29010 Málaga, SpainDepartment of Medicine and Dermatology, University of Málaga, Instituto de Investigación Biomédica de Málaga (IBIMA), 29010 Málaga, SpainPseudoxanthoma elasticum (PXE) is a genetic disease characterized by the calcification of elastin fibers. Our aim was to quantify vascular calcification in the arteries and the deposition of 18F-<i>sodium-fluoride</i> (18F-NaF) in the skin and vessel walls with positron emission tomography/computed tomography. This was an observational study including 18 patients with PXE. Vascular calcification was measured in Agatston units, and deposition in the skin and vessel walls was shown using target-to-background ratio (TBR). Severity of the disease was scored by Phenodex. We found higher vascular calcification in the popliteal, femoral, and aortic arch vessels compared to other vascular regions; however, the uptake of radiotracer was the highest in the aorta and femoral arteries. In the skin, the highest uptake was observed in the neck and the axillae. There was no significant association between 18F-NaF deposition in the arteries or skin and the global Phenodex score. In contrast, the Phenodex score was significantly associated in univariate analyses with the averaged vascular calcium score (<i>p</i> < 0.01). In the neck, patients with higher skin Phenodex scores exhibited higher radiotracer uptake. As a conclusion, because vascular calcification is physiological, our data suggested that the detection of cutaneous (neck) 18F-NaF deposits might serve to monitor the calcification process in the short-term for patients with PXE.https://www.mdpi.com/2077-0383/9/5/1393pseudoxanthoma elasticumPhenodexdisease activity18F-NaF PET/CT
spellingShingle Antonio Gutierrez-Cardo
Eugenia Lillo
Belén Murcia-Casas
Juan Luis Carrillo-Linares
Francisco García-Argüello
Purificación Sánchez-Sánchez
Alejandro Rodriguez-Morata
Isabel Baquero Aranda
Miguel Ángel Sánchez-Chaparro
María García-Fernández
Pedro Valdivielso
Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
Journal of Clinical Medicine
pseudoxanthoma elasticum
Phenodex
disease activity
18F-NaF PET/CT
title Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_full Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_fullStr Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_full_unstemmed Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_short Skin and Arterial Wall Deposits of 18F-NaF and Severity of Disease in Patients with Pseudoxanthoma Elasticum
title_sort skin and arterial wall deposits of 18f naf and severity of disease in patients with pseudoxanthoma elasticum
topic pseudoxanthoma elasticum
Phenodex
disease activity
18F-NaF PET/CT
url https://www.mdpi.com/2077-0383/9/5/1393
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