Gutierrez-Cardo Antonio, Lillo Eugenia, Murcia-Casas Belén, Carrillo-Linares Juan Luis, García-Argüello Francisco, Sánchez-Sánchez Purificación, Rodriguez-Morata Alejandro, Aranda Isabel Baquero, Sánchez-Chaparro Miguel Ángel, García-Fernández María, Valdivielso Pedro
Molecular Image Unit, Nuclear Medicine, Fundación General de la Universidad de Málaga, 29010 Málaga, Spain.
Department of Medicine and Dermatology, University of Málaga, Instituto de Investigación Biomédica de Málaga (IBIMA), 29010 Málaga, Spain.
J Clin Med. 2020 May 8;9(5):1393. doi: 10.3390/jcm9051393.
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-sodium-fluoride (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 ( < 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.
弹性假黄瘤（PXE）是一种以弹性纤维钙化为特征的遗传性疾病。我们的目的是通过正电子发射断层扫描/计算机断层扫描定量分析动脉中的血管钙化以及皮肤和血管壁中18F - 氟化钠（18F - NaF）的沉积情况。这是一项纳入18例PXE患者的观察性研究。血管钙化以阿加斯顿单位进行测量，皮肤和血管壁中的沉积情况通过靶本底比（TBR）显示。疾病严重程度采用Phenodex评分。我们发现，与其他血管区域相比，腘动脉、股动脉和主动脉弓血管中的血管钙化程度更高；然而，放射性示踪剂在主动脉和股动脉中的摄取量最高。在皮肤中，颈部和腋窝的摄取量最高。动脉或皮肤中18F - NaF沉积与整体Phenodex评分之间无显著关联。相比之下，在单因素分析中，Phenodex评分与平均血管钙化评分显著相关（<0.01）。在颈部，皮肤Phenodex评分较高的患者放射性示踪剂摄取量更高。总之，由于血管钙化是生理性的，我们的数据表明，检测皮肤（颈部）18F - NaF沉积可能有助于在短期内监测PXE患者的钙化过程。
