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Background: Ankle-brachial index (ABI) at rest, postexercise ABI, and toe-brachial index (TBI) are essential diagnostic tools recommended for peripheral artery disease (PAD) diagnosis. Our study investigates the level of knowledge on these 3 tests among vascular medicine residents from 4 French medical schools in France., Methods: We included 19 vascular medicine residents in a cross-sectional study. During an annual obligatory seminar, all residents accepted to fill 3 questionnaires concerning knowledge about these 3 tests., Results: All residents accepted to fill 3 questionnaires. None of the residents correctly knows how to perform all pressure measurements (ABI, postexercise ABI, and TBI). Two residents had the knowledge to perform the whole ABI at rest procedure, whereas no resident had the knowledge to perform neither the postexercise ABI (P = 0.48) nor the TBI (P = 0.48). Twelve residents correctly completed the question regarding the interpretation of ABI at rest, whereas 2 correctly completed the postexercise ABI question (P = 0.001) and 4 the TBI question (P = 0.02). The number of residents who have performed more than 20 measurements is higher regarding ABI at rest than postexercise ABI and TBI (84%, 5%, and 37% respectively; P < 0.001 and P = 0.006 respectively) and significantly less often in postexercise ABI than TBI (5% vs. 37%; P = 0.04)., Conclusions: This study shows for the first time that residents' knowledge of pressure measurements (resting-ABI, postexercise ABI, and TBI) of 4 French medical school are insufficient although the importance of pressure measurement has been strongly highlighted by the newly released PAD guidelines (2016) for PAD diagnosis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Spondylarthritis (SpA) is an inflammatory rheumatic disease associated with increased incidence of major adverse cardiovascular events (MACEs). Recently, Paramarta et al. proposed the use of the tyrosine kinase inhibitor Nilotinib in Spondyloarthritis to target certain inflammatory pathways. However, Nilotinib, which is highly effective for the treatment of patients with chronic myeloid leukaemia (CML), is also associated with an increased risk of MACEs. The authors suggest that Nilotinib may be effective in peripheral SpA by modulating inflammation, but not in axial SpA. Considering the vascular toxicity of Nilotinib and the acceleration of atherosclerosis in SpA patients, we suggest taking MACEs as an end-point in future trials.

Background and Aims: Pseudoxanthoma elasticum (PXE; OMIM 264800, prevalence 1/25,000 to 1/50,000) is an autosomal recessive multisystem disease due to deficiency in ABCC6, an ATP-binding cassette, sub-family C transporter. The PXE phenotype is mainly characterized by progressive ectopic calcification of connective tissues (namely skin, retinal Bruch's membrane and peripheral arteries) but the impact of PXE on bone structure is currently unknown. The present study sought to investigate bone mineralization and its potential link with vascular calcification in a large cohort of PXE patients with inherited mutations of the ABCC6 gene., Methods and Results: 96 patients (61 women) matching the PXE criteria participated in this study. Their clinical history and status and bone biological markers were collected. Bone mineral density (BMD) was measured by dual energy X-ray absorptiometry and expressed as T- and Z-scores. Osteoporotic fractures were identified by X-ray, and coronary (CAC) and lower limb arterial calcification (LLAC) scores were determined by CT scan., Results: 44% of the women were menopausal. Osteopenia was disclosed in 46% (17 women) while 23% (9 women) exhibited osteoporosis, 3 with severe osteoporosis. Fractures of an osteoporotic nature were authenticated in 3 patients (1 woman). Markers of bone remodelling processes (CTX, BSAP and osteocalcin) were within the normal range for our laboratory standards. Severe vitamin D deficiency (<25nmol/L) was found in 15%, while 51% exhibited no vitamin D deficiency (vitamin D≥50nmol/L). LLAC and CAC scores were significantly higher in the patients with a low T- and/or Z-score, although this difference disappeared in multivariate analysis with age as a confounding factor. There was no significant difference in LLAC and CAC between PXE patients with and without osteoporotic fractures. There was no statistically significant association between BMD, LLAC and CAC and any of the bone remodelling factors., Conclusions: This is the first report on the bone mineralization process in PXE patients. Our data shows that PXE patients are not markedly prone to exaggerated bone demineralization and fracture risk, and prevalence of osteoporosis remains within the normal range for the general population. Furthermore, the relationships between LLAC, but not CAC, and BMD with age are similar to those observed in the general population. Therefore, despite its pivotal role in ectopic calcification, ABCC6 deficiency does not interfere with the bone-vascular axis. The lack of PXE-related disturbances between BMD and arterial calcification also supports vitamin D supplementation in PXE patients with vitamin D deficiency. ClinicalTrials.gov Identifier: NCT01446393., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Rationale: Sildenafil, a phosphodiesterase-5-inhibitor and Bosentan, an endothelin-1-receptor antagonist combined therapy could have beneficial effect in systemic sclerosis (SSc) patients with peripheral artery disease., Patient Concerns: We report a case of a 48-year-old Black woman, who developed severe left limb claudication and walking limitation following a left femoropopliteal bypass occlusion in 2014. She was a heavy smoker and had a history of right middle cerebral artery ischemic stroke and bilateral Raynaud phenomenon., Diagnoses: According to the American College of Rheumatology/European League Against Rheumatism-2013 criteria, diagnosis of limited cutaneous SSc was retained with macrovascular lesions. She was referred for investigation of left limb claudication on treadmill using transcutaneous oxygen pressure measurement during exercise to argue for the vascular origin of the walking impairment. She had a severe left limb ischemia and the maximum walking distance (MWD) she reached was 118 m in March 2015 despite the medical optimal treatment and walking rehabilitation., Interventions: Sildenafil, 20 mg tid, was introduced due to active digital ulcers. In July 2015, the MWD increased to 288 m, then to 452 m in December 2015. Adding Bosentan to Sildenafil to prevent recurrent digital ulcers resulted in an MWD of 1576 m., Outcomes: Recently, the patient is treated with the combined therapy. She has no more pain during walking and his quality of life has improved., Lessons: Sildenafil and Bosentan combined therapy was associated in our case with an improvement of MWD without adverse effect. Further clinical trials are necessary to confirm our original observation.

Rationale: Sildenafil, a phosphodiesterase-5-inhibitor (PDE5i), could represent a new treatment in addition to the medical treatment and advice to walk in peripheral arterial disease (PAD)., Patient Concerns and Diagnoses: We report a case of a 62-year-old heavy smoker man who developed a buttock claudication and a severe walking limitation following an aorto-bi-femoral bypass in 1992. Since 2003, each year, he has been referred for investigation of bilateral buttock claudication on treadmill using transcutaneous oxygen pressure (tcpO2) measurement during exercise to argue for the vascular origin of the walking impairment. He had a severe bilateral buttock ischemia and the maximum walking distance (MWD) he reached was 258 m in 2011 despite the medical optimal treatment and walking rehabilitation. Ethical approval is not necessary for this case report according to the French legislation and written consent to publication was obtained from the patient., Interventions: Sildenafil, 100 mg/d, was introduced in February 2015 and the MWD increased to 310 m only after 2 h after the first oral intake, then to 713 m after 3 weeks, and finally to 1313 m in January 2017., Outcomes: Recently, the patient is treated with Sildenafil 100 mg/d. He has no more pain during walking and his quality of life has improved., Main Lessons to Learn: Sildenafil, a PDE5i, may represent a new therapeutic option in addition to the conventional optimal medical therapy in patients with arterial claudication. tcpO2 measurement during exercise is a promising technique for the diagnosis and monitoring of patients with PAD. A crossover, double-blind, prospective randomized monocenter study (ARTERIOFIL-NCT02832570) and a double-blind prospective randomized multicenter study (VALSTAR-NCT02930811) are ongoing to confirm our original observation.

Purpose: The "Walking Estimated-Limitation Calculated by History" (WELCH) questionnaire is a short four-item questionnaire that estimates the walking capacity of a patient in comparison with relatives, friends or people the same age. As such, it should be relatively insensitive to age, yet this has never been tested., Methods: A prospective study was performed among 525 patients presenting vascular-type claudication. Patients were grouped into quintiles of age-from quintile 1 for the youngest patients to quintile 5 for the oldest ones. Patients completed a self-administered questionnaire and then had their maximal walking time (MWT) measured on a treadmill. We estimated the coefficient of correlation, the slope and the intercept of the relationship between the WELCH score and the MWT, then the accuracy of a WELCH score under 25 to predict the ability to walk for 5 min on a treadmill., Results: The slopes of the relationships and the correlation coefficients were not significantly different in each quintile, but a significant shift in the intercept of regressing lines was found with age. Nevertheless, the accuracy in predicting treadmill results from the WELCH score with a cut-off point of 25 was, respectively, 68.6, 72.4, 80.0, 72.4 and 73.3 % in quintiles 1, 2, 3, 4 and 5 (p = 0.45)., Conclusions: The relationship of MWT on a treadmill and the WELCH score is slightly influenced by age, but a score superior to 25 seems to be of equal discriminatory performance in different quintiles of age to predict the ability to walk for 5 min on a treadmill. This makes this cut-off limit of interest for routine use, regardless of age.

Introduction: Laser speckle contrast imaging (LSCI) allows an easy non-contact monitoring of the cutaneous blood flow (CBF), but is highly sensitive to movement artifacts (ARTm). Subtraction of a signal recorded on an adhesive opaque surface (AOS) close to the area of interest was reported as a mean of reducing noise from the raw skin LSCI (LSCIsk) signal, provided an individual calibration was performed. Assuming that AOS=a·CBF+b·ARTm, an ideal patch should completely block the light reflection due to CBF and thus be insensitive to skin blood flow changes ("a"~0), while keeping a reflection signal amplitude similar to the one from the skin in case of artifact ("b"~1). This ideal AOS has not been determined and may discriminate flow from movements during LSCI recordings., Materials and Methods: We tested different AOSs to determine their "a" and "b" parameters in 35 and 34 healthy volunteers, respectively. The AOS surface providing results as close as possible to an ideal AOS, was used for a point-by-point de-noising of post occlusive reactive hyperemia (PORH) on two different days in 15 new subjects. Correlation of raw, smoothed (average smoothing over 1s intervals) and denoised signals was tested through a cross-correlation analysis of the two POHR tests., Results: The optimal "a" and "b" values were obtained with a homemade bilayer adhesive patch (a=0.06±0.05 and b=1.03±0.17) whereas other tested AOS had "a" values ranging from 0.05 to 0.23 and "b" values ranging from 2.69 to 3.82. Using the bilayer adhesive patch the cross-correlation between the two tests of POHR increased from 0.330±0.128 for raw, to 0.461±0.168 for smoothed and 0.649±0.128 for denoised signals respectively (p<0.05 from raw coefficients)., Conclusion: The home-made bilayer adhesive seems the optimal AOS for the removal of ARTm from the LSCIsk signal while respecting CBF signal. This specific AOS allows for an efficient de-noising of LSCI measurements without the need for individual calibration., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Vascular calcification is a complex and dynamic process occurring in various physiological conditions such as aging and exercise or in acquired metabolic disorders like diabetes or chronic renal insufficiency. Arterial calcifications are also observed in several genetic diseases revealing the important role of unbalanced or defective anti- or pro-calcifying factors. Pseudoxanthoma elasticum (PXE) is an inherited disease (OMIM 264800) characterized by elastic fiber fragmentation and calcification in various soft conjunctive tissues including the skin, eyes, and arterial media. The PXE disease results from mutations in the ABCC6 gene, encoding an ATP-binding cassette transporter primarily expressed in the liver, kidneys suggesting that it is a prototypic metabolic soft-tissue calcifying disease of genetic origin. The clinical expression of the PXE arterial disease is characterized by an increased risk for coronary (myocardial infarction), cerebral (aneurysm and stroke), and lower limb peripheral artery disease. However, the structural and functional changes in the arterial wall induced by PXE are still unexplained. The use of a recombinant mouse model inactivated for the Abcc6 gene is an important tool for the understanding of the PXE pathophysiology although the vascular impact in this model remains limited to date. Overlapping of the PXE phenotype with other inherited calcifying diseases could bring important informations to our comprehension of the PXE disease.