Increased Elastin Degradation in Pseudoxanthoma Elasticum Is Associated with Peripheral Arterial Disease Independent of Calcification.
Increased Elastin Degradation in Pseudoxanthoma Elasticum Is Associated with Peripheral Arterial Disease Independent of Calcification.
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DOI：
10.3390/jcm9092771
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发表时间：
2020-08-26
影响因子：
3.9
通讯作者：
de Jong PA
中科院分区：
文献类型：
--
作者：
Bartstra JW;Spiering W;van den Ouweland JMW;Mali WPTM;Janssen R;de Jong PA
弹性假黄瘤（PXE）导致外周动脉中弹性蛋白纤维广泛断裂和钙化，从而导致外周动脉疾病（PAD）。目前的研究主要集中在钙化在PXE发病机制中的作用。弹性蛋白降解和钙化被证明是相互作用的，并可能相互放大。本研究旨在比较PXE患者和对照组之间的血浆锁链素（弹性蛋白降解的一种指标），并研究锁链素与PXE中（1）动脉钙化，（2）PAD和（3）独立于动脉钙化的PAD之间的相关性。采用液相色谱-串联质谱法对93例PXE患者和72例对照者的血浆锁链素进行定量分析。在PXE患者中，动脉钙化块在CT扫描上被量化。活动平板试验后踝臂指数（ABI）< 0.9为PAD，方丹分型区分有症状和无症状PAD。建立回归模型，以检验链索蛋白与动脉钙化和动脉功能之间的关系。PXE患者具有比对照更高的锁链素（350（290-410）ng/L对320（280-360）ng/L，p = 0.02）。在调整了年龄、性别、体重指数、吸烟、2型糖尿病和肺部异常后，锁链素与更差的ABI相关（β（95%CI）：−68（−132; −3）ng/L）、更多PAD（β（95%CI）：40（7; 73）ng/L）和更高方丹分级（β（95%CI）：30（6; 53）ng/L），但不伴有动脉钙化肿块。较低的ABI与较高的锁链相关，与动脉钙化质量无关（β（95%CI）：-0.71（-1.39;-0.01））。与对照组相比，弹性蛋白降解在PXE患者中加速。锁链素和ABI之间的关联强调了弹性蛋白降解在PXE中PAD中的作用。我们的研究结果表明，弹性蛋白降解和动脉钙化独立有助于在PXE PAD。
Pseudoxanthoma elasticum (PXE) results in extensive fragmentation and calcification of elastin fibers in the peripheral arteries, which results in peripheral arterial disease (PAD). Current research focuses on the role of calcifications in the pathogenesis of PXE. Elastin degradation and calcification are shown to interact and may amplify each other. This study aims to compare plasma desmosines, a measure of elastin degradation, between PXE patients and controls and to investigate the association between desmosines and (1) arterial calcification, (2) PAD, and (3) PAD independent of arterial calcification in PXE. Plasma desmosines were quantified with liquid chromatography-tandem mass spectrometry in 93 PXE patients and 72 controls. In PXE patients, arterial calcification mass was quantified on CT scans. The ankle brachial index (ABI) after treadmill test was used to analyze PAD, defined as ABI < 0.9, and the Fontaine classification was used to distinguish symptomatic and asymptomatic PAD. Regression models were built to test the association between desmosines and arterial calcification and arterial functioning in PXE. PXE patients had higher desmosines than controls (350 (290–410) ng/L vs. 320 (280–360) ng/L, p = 0.02). After adjustment for age, sex, body mass index, smoking, type 2 diabetes mellitus, and pulmonary abnormalities, desmosines were associated with worse ABI (β (95%CI): −68 (−132; −3) ng/L), more PAD (β (95%CI): 40 (7; 73) ng/L), and higher Fontaine classification (β (95%CI): 30 (6; 53) ng/L), but not with arterial calcification mass. Lower ABI was associated with higher desmosines, independent from arterial calcification mass (β (95%CI): −0.71(−1.39; −0.01)). Elastin degradation is accelerated in PXE patients compared to controls. The association between desmosines and ABI emphasizes the role of elastin degradation in PAD in PXE. Our results suggest that both elastin degradation and arterial calcification independently contribute to PAD in PXE.
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