Ariane L Herrick

..Conversely, it is to be hoped that in the future, drugs shown to be effective in SSc might prove beneficial for other fibrotic diseases...

..To evaluate the effects of a combination of micronutrient antioxidants (selenium, beta-carotene, vitamin C, vitamin E and methionine) with allopurinol in patients with limited cutaneous systemic sclerosis (SSc)...

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..Finally we discuss management, including family screening, avoidance of triggering factors, analgesia, maintenance of a high calorie intake, and administration of haem derivatives...

..As our understanding of the pathophysiology of Raynaud's phenomenon increases, so do our possibilities for identifying effective treatments...

..The necessity of prompt assessment and treatment of acute digital ischemia, which is a medical emergency, is highlighted...

..Recent advances in genetic methodologies should further our understanding of this complex disease process...

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..Conversely a wide-field, high-magnification system that allows visualization of the whole nail bed offers a highly reproducible approach for quantitative assessment and therefore has potential as an outcome measure...

..v. vasodilators, debridements or amputations), (ii) a positive ACA, (iii) clinically evident calcinosis, (iv) pulmonary arterial hypertension and (v) telangiectases...

..Our aim was to compare semi-automatic feature measurement from newly developed software with manual measurements, and determine the degree to which semi-automated data allows disease group classification...

..Good reliability is a necessary prerequisite for a test to be a valid clinical or research tool...

..Our aim was to describe clinical features and pattern of care in children with localized scleroderma presenting to secondary care during a 25-month incidence study...

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..New approaches to therapy are being driven by increased understanding of pathophysiology and by increased international networking of clinicians and scientists, facilitating clinical trials...

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..Topical, or transdermal, treatments that increase digital blood flow without systemic adverse effects are, therefore, an attractive option. A novel topical nitroglycerin formulation has shown promise in treating Raynaud attacks...

..If reproducibility is confirmed to be satisfactory, then these techniques could be used to examine disease progression over time and responsiveness to vasoactive treatment, thus facilitating clinical trials...

..This review will discuss diagnosis, subtyping, and the major clinical features and their management...

..Therefore one technique cannot substitute for another...

..Our aim was to ascertain the incidence of childhood scleroderma in its different forms in the UK and Ireland, and to describe the age, sex, and ethnicity of the cases...

..The current treatment of choice for scleroderma-related critical digital ischaemia and/or ulceration is intravenous prostanoid therapy, necessitating hospitalisation. However, iloprost is often poorly tolerated and may be ineffective...

..For each of these, the method(s) and then the clinical and research applications are discussed. Laser Doppler, a research technique, is also described...

..Laser Doppler imaging (LDI) of perfusion has been performed with a novel green wavelength (532 nm) for comparison with a HeNe laser (633 nm), the aim being validation of the green laser wavelength as a research tool...

..However, in contrast to previous studies, we found no specific pattern of a morphological nature of nailfold capillaries in patients with psoriasis with or without nail changes, when compared with normal controls...

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..As well as demonstrating the effectiveness of topical GTN in patients with PRP and LCSSc, this study illustrates the ability of laser Doppler imaging to quantify local vasodilatory effects...

..This study investigated whether whole finger vasodilator iontophoresis increases digital blood flow in patients with systemic sclerosis (SSc): If so, this might indicate a novel approach to therapy...

..To test the intra- and interobserver variability, among clinicians with an interest in systemic sclerosis (SSc), in defining digital ulcers...

..There were no associations with psychosocial variables. CONCLUSION: RP is frequent in children ages 12-15 years. Unlike other common childhood symptoms, RP showed no association with psychosocial factors in this study...

..Bone mineral density (BMD) can now be measured accurately using dual energy x ray absorptiometry (DEXA). Recently, DEXA has been used to measure periarticular BMD at predefined regions of interest (ROIs) around the joints...

..Increased blood flow occurs in plaques of psoriasis, and an increase in blood flow has been shown to occur in uninvolved skin adjacent to the active edge...

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..The potential association with severe calcinosis merits further study. Prospective studies are required to investigate acro-osteolysis as a marker of digital vascular disease progression and of treatment response...

..To examine patients' beliefs about systemic sclerosis (SSc) and to investigate the relationship between these beliefs, symptom report, and clinical and demographic variables...

..To examine the effect of sildenafil in patients with Raynaud's phenomenon (RP) secondary to limited cutaneous systemic sclerosis (lcSSc)...

..Cigarette smoking is an important risk factor for vascular disease in the general population. We investigated the influence of cigarette smoking on digital ischemia in patients with SSc...

..Increased GLUT-1 expression in lcSSc/cal and dcSSc indicates that hypoxia is an associated factor...

..Dual-wavelength LDI may help predict treatment response...

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..Prospective longitudinal studies are required to investigate whether anti-oxidized LDL antibodies may be a marker of vascular damage in SSC...

..Care of the feet is therefore an important part of the management of SSC...

..04 (95% CI -0.01 to +0.09). CONCLUSIONS: The severity of large vessel macrovascular disease, as assessed by ABPI, is not dependent on disease subtype. Anticentromere antibody may be weakly associated with a reduction in ABPI...

..Little is known about the pathophysiology of localized scleroderma (skin fibrosis, also termed 'morphoea'), although it is likely that microvascular dysfunction is a contributing factor...

..The nodules failed to respond to therapy with topical steroid, calcipotriol, or extended photochemotherapy. This is a rare, disfiguring variant of scleroderma, unresponsive to treatment, and poorly understood...

..92 for maximum thickness). CONCLUSION: Our results suggest that the 17-point dermal ultrasound scoring system is extremely reliable and may therefore be a useful measure of outcome, including in clinical trials...

..However, there may have been some radiographic improvement. Given that there is currently no effective treatment for SSc-related calcinosis a larger study incorporating higher 'doses' of iontophoresis is indicated...

..We undertook an association study to explore the role of COL15 single-nucleotide polymorphisms (SNP) in SSc disease development...

..Therapy was well tolerated with no increase in serious adverse events...

..To develop a provisional core set of response measures for clinical trials of systemic sclerosis (SSc)...

..Additionally, we describe a de novo heterozygous mutation, affecting a critical catalytic residue in TREX1, that results in typical Aicardi-Goutieres syndrome...