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This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1. [ABSTRACT FROM AUTHOR]

Background: Priority setting, identification of unmet and changing healthcare needs, service and policy planning, and the capacity to evaluate the impact of health interventions requires valid and reliable methods for quantifying disease and injury burden. The methodology developed for the Global Burden of Disease (GBD) studies has been adopted to estimate the burden of disease in national, regional and global projects. However, there has been little validation of the methods for estimating injury burden using empirical data. Objective: To provide valid estimates of the burden of non-fatal injury using empirical data. Setting: Data from prospective cohort studies of injury outcomes undertaken in the UK, USA, Australia, New Zealand and The Netherlands. Design and participants: Meta-analysis of deidentified, patient-level data from over 40 000 injured participants in six prospective cohort studies: Victorian State Trauma Registry, Victorian Orthopaedic Trauma Outcomes Registry, UK Burden of Injury study, Prospective Outcomes of Injury study, National Study on Costs and Outcomes of Trauma and the Dutch Injury Patient Survey. Analysis: Data will be systematically analysed to evaluate and refine injury classification, development of disability weights, establishing the duration of disability and handling of cases with more than one injury in burden estimates. Developed methods will be applied to incidence data to compare and contrast various methods for estimating non-fatal injury burden. Contribution to the field: The findings of this international collaboration have the capacity to drive how injury burden is measured for future GBD estimates and for individual country or region-specific studies. [ABSTRACT FROM AUTHOR]

Objective To delineate the constitutive pulmonary vascular phenotype of the TβRIIΔk-fib mouse model of scleroderma, and to selectively induce pulmonary endothelial cell injury using vascular endothelial growth factor (VEGF) inhibition to develop a model with features characteristic of pulmonary arterial hypertension (PAH). Methods The TβRIIΔk-fib mouse strain expresses a kinase-deficient transforming growth factor β (TGFβ) receptor type II driven by a fibroblast-specific promoter, leading to ligand-dependent up-regulation of TGFβ signaling, and replicates key fibrotic features of scleroderma. Structural, biochemical, and functional assessments of pulmonary vessels, including in vivo hemodynamic studies, were performed before and following VEGF inhibition, which induced pulmonary endothelial cell apoptosis. These assessments included biochemical analysis of the TGFβ and VEGF signaling axes in tissue sections and explanted smooth muscle cells. Results In the TβRIIΔk-fib mouse strain, a constitutive pulmonary vasculopathy with medial thickening, a perivascular proliferating chronic inflammatory cell infiltrate, and mildly elevated pulmonary artery pressure resembled the well-described chronic hypoxia model of pulmonary hypertension. Following administration of SU5416, the pulmonary vascular phenotype was more florid, with pulmonary arteriolar luminal obliteration by apoptosis-resistant proliferating endothelial cells. These changes resulted in right ventricular hypertrophy, confirming hemodynamically significant PAH. Altered expression of TGFβ and VEGF ligand and receptor was consistent with a scleroderma phenotype. Conclusion In this study, we replicated key features of systemic sclerosis-related PAH in a mouse model. Our results suggest that pulmonary endothelial cell injury in a genetically susceptible mouse strain triggers this complication and support the underlying role of functional interplay between TGFβ and VEGF, which provides insight into the pathogenesis of this disease. [ABSTRACT FROM AUTHOR]

Objectives. To define the clinical, serological, histological and immunogenetic features of patients with scleroderma and ANCA-associated vasculitis (AAV).Methods. We examined a clinical database of 2200 patients with either limited or diffuse cutaneous systemic sclerosis (SSc). Patients with a confirmed diagnosis of vasculitis who were ANCA positive with either MPO or PR3 reactivity had their clinical features, serology, histology and HLA haplotypes examined in detail.Results. From this SSc cohort, 35 patients (1.6%) had evidence of vasculitis, and the SSc autoantibody profiles in this group were comparable to those previously published from the whole cohort. Of these 35 patients, 8 (0.4% of whole SSc cohort) had either anti-MPO or anti-PR3 antibodies and two further patients were ANCA positive without defined specificities. Of the eight ANCA-positive patients, seven had limited cutaneous SSc and anti-MPO antibodies and only one had anti-PR3 antibodies, associated with diffuse cutaneous SSc. Two ANCA-positive patients had anti-U3RNP antibodies, usually associated with overlap disease. None of the patients had granulomatous disease. The majority had glomerulonephritis, renal arteritis and pulmonary fibrosis. There were several shared HLA haplotypes from the DP and DQ loci in these overlap patients.Conclusion. SSc in overlap with ANCA-associated vasculitis is rare, and clinical features are more mixed than when either of these two conditions occurs separately. From our database, U3RNP antibodies may be more associated with overlap AAV than the other scleroderma-specific antibodies. [ABSTRACT FROM AUTHOR]

Background: Methadone is a synthetic, narcotic analgesic used in the treatment of drug misuse. Tragedies involving children being poisoned by the accidental ingestion of methadone are no longer a rare occurrence. Following an audit of the effectiveness of the provision and recall of information to patients attending an NHS Methadone Clinic a protocol was introduced to ensure that staff documented the provision of such information and patients gave a written confirmation that they had received the information. Methods: The study was undertaken in the setting of an NHS methadone clinic with the aim of re-auditing the storage of methadone at home following the introduction of the new protocols. 174 patients completed an anonymous questionnaire regarding where they store methadone at home and whether they recall being given advice about safe storage. Community pharmacists were contacted by telephone to assess the level of advice given to methadone patients regarding safety. Results: Only 49 (28.2%) patients recalled being given advice about safe storage, 24 (13.8%) recalled that information was provided by clinic staff. 170 (97.7%) patients regard methadone as being dangerous. (28.2%). Methadone is most commonly stored in a cupboard (37.9%). All methadone is dispensed in a bottle with a child resistant cap on it. All patients reported they stored their methadone in the original bottle provided by the pharmacist. Conclusion: Recall of information on safety issues is very poor. Provision of written as well as verbal information is needed. The use of printed safety information cards which patients can take away for future reference may be of use. It is the responsibility of health professionals to ensure they provide information and advice to methadone users on the safe storage of their methadone at home. [ABSTRACT FROM AUTHOR]

This article considers the results of a retrospective study comparing pregnancy rates in one Drug Testing and Treatment Order (DTTO) service in the United Kingdom with pregnancy rates in a generic substance misuse service and national conception rates. Conception rates were found to be six times higher in the coerced drug treatment group compared to the general population and generic drug services over the same period. These higher pregnancy rates may possibly be accounted for by an interplay of several factors including improved physical health, improvements in menstrual cycle regularity, better social interactions or intrinsic nature of the treatment regime in the clinics. The results of the study have implications for the criminal justice, health and other services involved in the care of these patients. [ABSTRACT FROM AUTHOR]

The article presents the second part of a series of articles about the British National Health Service (NHS). In this study, researchers describe moves toward explicit prioritization of patients in other countries, particularly New Zealand. Experiences suggest new directions for the NHS and pitfalls to avoid. New Zealand introduces a nation-wide system for explicitly prioritizing and booking patients for elective surgery.

The article discusses on the National Health Service's (NHS) waiting list management plan in Great Britain. The difficulties in target implementation and management, fundamental issues of equity and efficiency are not focused on waiting number and number targets of the NHS Plan. This target cannot guarantee the equity of the people with the same condition in different areas and people with different conditions in the same area.

The article presents an editorial looking at the altered roles of primary care nurses and health visitors, as well as their relationship with general practitioners. The article discusses Great Britain's National Health Service (NHS) walk-in centers, NHS Direct, why nurses and health visitors are withdrawing from primary health care teams in England, and health promotion.