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      The Contribution of Arterial Calcification to Peripheral Arterial Disease in Pseudoxanthoma Elasticum

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          Abstract

          Background and aims

          The contribution of arterial calcification (AC) in peripheral arterial disease (PAD) and arterial wall compressibility is a matter of debate. Pseudoxanthoma elasticum (PXE), an inherited metabolic disease due to ABCC6 gene mutations, combines elastic fiber fragmentation and calcification in various soft tissues including the arterial wall. Since AC is associated with PAD, a frequent complication of PXE, we sought to determine the role of AC in PAD and arterial wall compressibility in this group of patients.

          Methods and Results

          Arterial compressibility and patency were determined by ankle-brachial pressure index (ABI) in a cohort of 71 PXE patients (mean age 48±SD 14 yrs, 45 women) and compared to 30 controls without PAD. Lower limb arterial calcification (LLAC) was determined by non-contrast enhanced helicoidal CT-scan. A calcification score (Ca-score) was computed for the femoral, popliteal and sub-popliteal artery segments of both legs. Forty patients with PXE had an ABI<0.90 and none had an ABI>1.40. LLAC increased with age, significantly more in PXE subjects than controls. A negative association was found between LLAC and ABI (r = −0.363, p = 0.002). The LLAC was independently associated with PXE and age, and ABI was not linked to cardiovascular risk factors.

          Conclusions

          The presence of AC was associated with PAD and PXE without affecting arterial compressibility. PAD in PXE patients is probably due to proximal obstructive lesions developing independently from cardiovascular risk factors.

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          Most cited references39

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          Ankle-arm index as a marker of atherosclerosis in the Cardiovascular Health Study. Cardiovascular Heart Study (CHS) Collaborative Research Group.

          D Siscovick, Stuart A. Newman, Jacquelyn L. Fried … (1993)
          Peripheral arterial disease measured noninvasively by the ankle-arm index (AAI) is common in older adults, largely asymptomatic, and associated with clinically manifest cardiovascular disease (CVD). The criteria for an abnormal AAI have varied in previous studies. To determine whether there is an inverse dose-response relation between the AAI and clinical CVD, subclinical disease, and risk factors, we examined the relation of the AAI to cardiovascular risk factors, other noninvasive measures of subclinical atherosclerosis using carotid ultrasound, echocardiography and electrocardiography, and clinical CVD. The AAI was measured in 5084 participants > or = 65 years old at the baseline examination of the Cardiovascular Health Study. All subjects had detailed assessment of prevalent CVD, measures of cardiovascular risk factors, and noninvasive measures of disease. Participants were stratified by baseline clinical CVD status and AAI ( or = 0.8 to or = 0.9 to or = 1.0 to < 1.5). Analyses tested for a dose-response relation of the AAI with clinical CVD, risk factors, and subclinical disease. The cumulative frequency of a low AAI was 7.4% of participants < 0.8, 12.4% < 0.9, and 23.6% < 1.0. participants with an AAI < 0.8 were more than twice as likely as those with an AAI of 1.0 to 1.5 to have a history of myocardial infarction, angina, congestive heart failure, stroke, or transient ischemic attack (all P < .01). In participants free of clinical CVD at baseline, the AAI was inversely related to history of hypertension, history of diabetes, and smoking, as well as systolic blood pressure, serum creatinine, fasting glucose, fasting insulin, measures of pulmonary function, and fibrinogen level (all P < .01). Risk factor associations with the AAI were similar in men and women free of CVD except for serum total and low-density lipoprotein cholesterol, which were inversely associated with AAI level only in women. Risk factors associated with an AAI of < 1.0 in multivariate analysis included smoking (odds ratio [OR], 2.55), history of diabetes (OR, 3.84), increasing age (OR, 1.54), and nonwhite race (OR, 2.36). In the 3372 participants free of clinical CVD, other noninvasive measures of subclinical CVD, including carotid stenosis by duplex scanning, segmental wall motion abnormalities by echocardiogram, and major ECG abnormalities were inversely related to the AAI (all P < .01). There was an inverse dose-response relation of the AAI with CVD risk factors and subclinical and clinical CVD among older adults. The lower the AAI, the greater the increase in CVD risk; however, even those with modest, asymptomatic reductions in the AAI (0.8 to 1.0) appear to be at increased risk of CVD.
          Article 0 comments Cited 135 times – based on 0 reviews     Review now
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            Media calcification and intima calcification are distinct entities in chronic kidney disease.

            Kerstin Amann (2008)
            Calcification of the vascular tree is common in physiologic and pathologic conditions, i.e., aging, diabetes, dyslipidemia, genetic diseases, and diseases with disturbances of calcium metabolism. In chronic kidney disease, vascular calcification is even more common, develops early, and contributes to the markedly increased cardiovascular risk in this particular population. Pathomorphologically, atherosclerosis (i.e., plaque-forming degenerative changes of the aorta and of large elastic arteries) and arteriosclerosis (i.e., concentric media thickening of muscular arteries) can be distinguished. Increasing knowledge about calcification together with improved imaging techniques provided evidence that also vascular calcification has to be divided into two distinct entities according to the specific sites of calcification within the vascular wall: Patchy calcification of the intima in the vicinity of lipid or cholesterol deposits as present in plaque calcification and calcification of the media in the absence of such lipid or cholesterol deposits, known as Mönckeberg-type atherosclerosis. The two types of calcification may vary according to the type of vessel (large elastic versus smaller muscular type artery) and proximal versus distal sites of the arterial tree. Furthermore, clinical studies showed that it is not purely academic to distinguish between intimal and medial calcification but rather relevant for the clinical presentation, treatment, and prognosis because each type leads to different clinical consequences. In vivo studies in animal models provided evidence in favor of common pathomechanisms between vascular calcification and atherosclerosis; however, there is other, strong experimental and clinical evidence that pleads for the continued distinction between intimal and medial calcification.
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              Medial localization of mineralization-regulating proteins in association with Mönckeberg's sclerosis: evidence for smooth muscle cell-mediated vascular calcification.

              D Proudfoot, Roger P Weissberg, Troy R. Cary … (1999)
              Calcification of the media of peripheral arteries is referred to as Mönckeberg's sclerosis (MS) and occurs commonly in aged and diabetic individuals. Its pathogenesis is unknown, but its presence predicts risk of cardiovascular events and leg amputation in diabetic patients. Several studies have documented expression of bone-associated genes in association with intimal atherosclerotic calcification, leading to the suggestion that vascular calcification may be a regulated process with similarities to developmental osteogenesis. Therefore, we examined gene expression in vessels with MS to determine whether there was evidence for a regulated calcification process in the vessel media. In situ hybridization, immunohistochemistry, and semiquantitative reverse-transcription polymerase chain reaction were used to examine the expression of mineralization-regulating proteins in human peripheral arteries with and without MS. MS occurred in direct apposition to medial vascular smooth muscle cells (VSMCs) in the absence of macrophages or lipid. These VSMCs expressed the smooth muscle-specific gene SM22alpha and high levels of matrix Gla protein but little osteopontin mRNA. Compared with normal vessels, vessels with MS globally expressed lower levels of matrix Gla protein and osteonectin, whereas alkaline phosphatase, bone sialoprotein, bone Gla protein, and collagen II, all indicators of osteogenesis/chondrogenesis, were upregulated. Furthermore, VSMCs derived from MS lesions exhibited osteoblastic properties and mineralized in vitro. These data indicate that medial calcification in MS lesions is an active process potentially orchestrated by phenotypically modified VSMCs.
              Article 0 comments Cited 100 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Philipp Eller: Role: Editor
                Journal
                Journal ID (nlm-ta): PLoS One
                Journal ID (iso-abbrev): PLoS ONE
                Journal ID (publisher-id): plos
                Journal ID (pmc): plosone
                Title: PLoS ONE
                Publisher: Public Library of Science (San Francisco, USA )
                ISSN (Electronic): 1932-6203
                Publication date Collection: 2014
                Publication date (Electronic): 6 May 2014
                Volume: 9
                Issue: 5
                Electronic Location Identifier: e96003
                Affiliations
                [1 ]PXE Health and Research Center, University Hospital of Angers, Angers, France
                [2 ]UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France
                [3 ]Department Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii, United States of America
                [4 ]UPRES 3860, School of Medicine, l'UNAM University, Angers, France
                [5 ]Clinical Research Center, University Hospital of Angers, Angers, France
                Medical University of Graz, Austria
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Conceived and designed the experiments: GL LM GK. Performed the experiments: GL LM SW. Analyzed the data: GL LM JFH. Contributed reagents/materials/analysis tools: DH JFH SW. Wrote the paper: GL GK PA LM. Drafted and revised the manuscript: DH OLS.

                Article
                Publisher ID: PONE-D-14-06674
                DOI: 10.1371/journal.pone.0096003
                PMC ID: 4011742
                PubMed ID: 24800819
                SO-VID: c8a10349-6480-477e-b477-8bdda0d81c9c
                Copyright statement: Copyright @ 2014
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 13 February 2014
                Date accepted : 1 April 2014
                Page count
                Pages: 7
                Funding
                French Society of Vascular Medicine (SFMV), University Hospital of Angers (contract # 2010-A00142-37 and #2011-A00942-39) and the PXE-France Patients' Association. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Subject: Research Article
                Subject: Biology and Life Sciences
                Subject: Genetics
                Subject: Genetic Dominance
                Subject: Autosomal Recessive Traits
                Subject: Medicine and Health Sciences
                Subject: Cardiology
                Subject: Clinical Genetics
                Subject: Dermatology
                Subject: Dermatologic Pathology
                Subject: Ophthalmology
                Subject: Inherited Eye Disorders
                Subject: Macular Disorders
                Subject: Rheumatology
                Subject: Connective Tissue Diseases
                Subject: Vascular Medicine
                Subject: Vascular Diseases
                Subject: Peripheral Vascular Disease
                Subject: Research and Analysis Methods
                Subject: Research Design
                Subject: Clinical Research Design
                Subject: Cohort Studies
                Subject: Cross-Sectional Studies
                Subject: Prospective Studies

                ScienceOpen disciplines: Uncategorized
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                ScienceOpen disciplines: Uncategorized

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