Abstract Objectives The purpose of this study was to assess health-related quality of life (HRQoL) and disease perception in a large, international group of patients with systemic sclerosis (SSc). ...Methods We placed a standardized questionnaire on a website for patient access. Socio-demographic information, disease characteristics and self-assessment questionnaires — the Short Form 36 (SF-36) and the Revised Illness Perception Questionnaire (IPQ-R) — were collected. Results 1902 patients from 60 countries were included. HRQoL appeared to be impaired in SSc, particularly for physical health (PCS, mean±SD 43.4±23.4). SSc patients also had strongly perceptions about the chronic nature and negative consequence of the disease, and experienced negative emotions due to SSc. Patients with diffuse cutaneous SSc had a poorer HRQoL than those with limited cutaneous SSc, for both physical (PCS, mean±SD 46.6±23.7 vs. 39.8±22.3; p <0.0001) and mental components (MCS, mean ± SD 53.8±23.0 vs. 50.3±23.2; p =0.003). Late-stage SSc patients were more likely to perceive their disease chronic ( p <0.0001), less controllable ( p =0.03) and with more consequences ( p =0.008), but they had a better understanding of their disease and experienced fewer negative emotions. Raynaud′s phenomenon and gastrointestinal complications were the organ involvement with the greatest impact on QoL, associated with the most negative perception of illness severity. Conclusion This study, performed on the largest group ever set up for this purpose, confirms the major impact on QoL and the negative perceptions of their disease expressed by SSc patients. However, the perception of this illness tended to improve with disease duration, suggesting that patients find effective coping strategies.
Systemic sclerosis (systemic scleroderma) is characterized by a heterogeneous range of clinical manifestations. Systemic sclerosis is classified into limited cutaneous systemic sclerosis and diffuse ...cutaneous systemic sclerosis subgroups based on the extent of skin involvement. Randomized controlled trials in scleroderma have mainly focused on diffuse cutaneous systemic sclerosis partly because the measurement of skin involvement, critical for evaluating a therapeutic intervention, is more dynamic in this subset. Nonetheless, limited cutaneous systemic sclerosis, the most common cutaneous subset (about two-third), is also associated with significant morbidity and detrimental impact on health-related quality of life. The lack of interventional studies in limited cutaneous systemic sclerosis is partly due to a lack of relevant outcome measures to evaluate this subgroup. Combining several clinically meaningful outcomes selected specifically for limited cutaneous systemic sclerosis may improve representativeness in clinical trials and responsiveness of outcomes measured in randomized controlled trials. A composite index dedicated to limited cutaneous systemic sclerosis combining such relevant outcomes could advance clinical trial development for limited cutaneous systemic sclerosis by providing the opportunity to test and select among candidate drugs that could act as disease-modifying treatments for this neglected subgroup of systemic sclerosis. This proposed index would include items selected by expert physicians and patients with limited cutaneous systemic sclerosis across domains grounded in the lived experience of limited cutaneous systemic sclerosis. This article reviews the reasons behind the relative neglect of limited cutaneous systemic sclerosis, discusses the current state of outcome measures for limited cutaneous systemic sclerosis, identifies challenges, and proposes a roadmap for a combined limited cutaneous systemic sclerosis-specific treatment response index.
Abstract When the diagnosis of systemic sclerosis (SSc) is made and you are told “ You have SSc ”, it is a strange feeling for the patient, because you don't yet know what it is exactly. It is a very ...intense shock to hear this, and it is also difficult to try to imagine what will follow. There is no cure for SSc; there are just treatments that are capable of reducing the symptoms. The main difficulties and pitfalls encountered by the patients occur: before the diagnosis; during treatment; at the hospital and with the physicians; if surgery is necessary; at home; with family and friends… How do you live with SSc? You need strong, active support to help you to live with this illness; without this, we cannot handle it. The way to communicate, to inform, to consider this disease is changing, because times are changing, patients are changing, and the opinions of the specialists are also changing. Of course it's a long way, but what we, the patients, hope for is to be better understood by you, the doctors. Please pay attention to what we say; we're the ones suffering from this disease. SSc is part of us; we are not merely medical cases! Please think about it. Together we can help you to fight, to find out more, to find a cure.
Abstract
Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two ...main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.
Objectives:
The objectives of this study were to describe the impact of systemic sclerosis associated interstitial lung disease, on quality of life, to estimate the correlation between quality of ...life and severity of lung disease and to assess the impact of interstitial lung disease on caregivers.
Methods:
Seven investigators included systemic sclerosis associated interstitial lung disease patients from December 2019 to April 2020. Sociodemographics and clinical data were collected. Patients reported outcomes and questionnaires were used with 1 generic patients reported outcome (EQ-5D-5L), 1 specific PRO (Brief Interstitial Lung Disease) and 2 self-reported questionnaires on impact of SSc complications and impact on caregivers. The correlation between forced vital capacity and EQ-5D-5L score was estimated with a multivariate linear regression model adjusted on several covariates.
Results:
In all, 89 patients were included. 26.4% were males, mean age was 58.2 ± 14.5 years. Mean EQ-5D-5L score = 0.79 ± 0.22 (median = 0.85). Mean EQ-5D-5L visual analog scale score = 60.8 ± 20.4 (median = 61.5). Mean King’s Brief Interstitial Lung Disease score = 58.4 ± 12.7 (median = 58.0). After adjustment on covariates, a significant correlation between forced vital capacity and EQ-5D-5L score was found with an increase of 0.003 of the EQ-5D-5L score for a 1% increase of FVC (p = 0.0096). No significant correlation between forced vital capacity and the EQ-VAS and King’s Brief Interstitial Lung Disease score were found. The impact of SSc on other organs was significantly correlated with EQ- 5D-5L score, respectively, for the impact scores on the lung system (p = 0.0003), heart system (p = 0.0182), Raynaud’s syndrome (p = 0.0015), digestive system (p = 0.0032), joints/muscles (p = 0.0003), skin (p < 0.0001), kidney (p = 0.0052) and gastro-oesophageal reflux (p = 0.0063). Significant correlations between King’s Brief Interstitial Lung Disease score and lung system (p < 0.0001), heart system (p < 0.0001), digital ulcers (p = 0.058), digestive system (p < 0.0001), kidney (p = 0.0004), skin (p = 0.0499) and gastro-oesophageal reflux (p = 0.0033) scores were found 68.5% of patients reported their need for a caregiver to help them in their daily life activities.
Conclusion:
Our study highlighted the strong burden of systemic sclerosis associated interstitial lung disease` for patients, especially with an impact on quality of life, on other organs manifestations and need for caregivers in their daily life.
Involving patients and health care professionals in research has been shown to be a useful strategy to generate program development ideas and establish research priorities. Online surveys can be used ...to identify patient research priorities. The Scleroderma Patient-centered Intervention Network is an international collaboration dedicated to researching problems important to people with scleroderma and developing, testing, and disseminating educational, self-management, rehabilitation, and psychological tools to support coping within the disease. The objectives of the Scleroderma Patient-centered Intervention Network Scleroderma Research Topics Survey were to (1) obtain input on online programs that Scleroderma Patient-centered Intervention Network could develop, (2) identify research topics important to members of the scleroderma community that could be addressed via the Scleroderma Patient-centered Intervention Network Cohort, and (3) identify potentially understudied groups. Eligible survey respondents included individuals with scleroderma and their caregivers, health care professionals, and patient organization representatives. Responses were evaluated separately for online programs, possible research questions, and understudied groups. In each category, thematic analysis was used to group responses into themes. A total of 125 participants (100 patients, 24 health care professionals, and 1 caregiver) completed the survey, and responses from patients and health care professionals were reported. For online programs, frequent recommendations included Interactive Platforms to facilitate communication between patients, as well as interventions targeting physical activity, and emotions and stress. Common responses for possible research questions were related to calcinosis, accessing health care, and quality of life. The most common suggestions for understudied groups were young patients, minority patients, and patients with calcinosis. Research topic suggestions provided in the survey will inform Scleroderma Patient-centered Intervention Network priorities and can be considered by other researchers.
Conventional equalization and carrier recovery algorithms for minimizing mean-square error in digital communication systems generally require an initial training period during which a known data ...sequence is transmitted and properly synchronized at the receiver. This paper solves the general problem of adaptive channel equalization without resorting to a known training sequence or to conditions of limited distortion. The criterion for equalizer adaptation is the minimization of a new class of nonconvex cost functions which are shown to characterize intersymbol interference independently of carrier phase and of the data symbol constellation used in the transmission system. Equalizer convergence does not require carrier recovery, so that carrier phase tracking can be carried out at the equalizer output in a decision-directed mode. The convergence properties of the self-recovering algorithms are analyzed mathematically and confirmed by computer simulation.
The performance of conventional modem receivers, where adaptive equalization is achieved by a digital transversal filter with tap gains spaced at the symbol interval, depends critically on the choice ...of the sampling phase. In this paper, a digital timing recovery loop is described and analyzed in the case of passband quadrature amplitude modulated data signals. Under conditions likely to be encountered on actual voiceband communication channels, the clock phase derived is shown to prevent spectral nulls and to accurately approximate the optimum timing phase for an infinite equalizer. Computer simulations show that the proposed system is capable of fast timing acquisition.
A 2400-Bit/s Microprocessor-Based Modem Godard, Dominique; Pilost, Daniel
IBM journal of research and development,
1/1981, Volume:
25, Issue:
1
Journal Article
Combining voice and data switched services into one network should lower costs, improve service, and ease introduction of new services. The architecture and implementation of transport and switching ...mechanisms are examined for future wide-area integrated private networks. These networks should satisfy requirements involving: 1. connection availability, 2. message integrity, 3. acceptable delay, 4. voice quality, 5. implementation and management complexity, 6. level of trunk utilization, 7. support of different bit rates, 8. connect/disconnect speed, 9. flexibility, and 10. compatibility with present technology. Fast packet switching should satisfy future requirements better than hybrid switching because of: 1. lower node implementation and control complexity, 2. easier availability enhancement, 3. shorter call setup time, and 4. greater line speed flexibility. However, hybrid switching offers shorter propagation time and better trunk utilization. Illustrations.
PDF
