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BACKGROUND Civilians are often first-line responders in hemorrhage control; however, windlass tourniquets are not intuitive. Untrained users reading enclosed instructions failed in 38.2% of tourniquet applications. This prospective follow-up study replicated testing following Stop the Bleed (STB) training. MATERIALS AND METHODS One and six months following STB, first-year medical students were randomly assigned a windlass tourniquet with enclosed instructions. Each was given one minute to read instructions and two minutes to apply the windlass tourniquet on the TraumaFX HEMO trainer. Demographics, time to read instructions and stop bleeding, blood loss, and simulation success were analyzed. RESULTS 100 students received STB training. 31 and 34 students completed tourniquet testing at one month and six months, respectively. At both intervals, 38% of students were unable to control hemorrhage (P = 0.97). When compared to the pilot study without STB training (median 48 sec, IQR 33-60 sec), the time taken to read the instructions was shorter one month following STB (P

Background Despite significant attempts to educate civilians in hemorrhage control, the majority remain untrained. We sought to determine if laypersons can successfully apply one of three commercially available tourniquets; including those endorsed by the United States Military and the American College of Surgeons. Methods Preclinical graduate health science students were randomly assigned a commercially available windless tourniquet: SAM XT, Combat Application Tourniquet (CAT), or Special Operation Forces Tactical Tourniquet (SOFT-T). Each was given up to 1 minute to read package instructions and asked to apply it to the HapMed Leg Tourniquet Trainer. Estimated blood loss was measured until successful hemostatic pressure was achieved or simulated death occurred from exsanguination. Simulation survival, time to read instructions and stop bleeding, tourniquet pressure, and blood loss were analyzed. Results Of the 150 students recruited, 55, 46, and 49 were randomized to the SAM XT, CAT, SOFT-T, respectively. Mean overall simulation survival was less than 66% (65%, 72%, 61%; p = 0.55). Of survivors, all three tourniquets performed similarly in median pressure applied (319, 315, and 329 mm Hg; p = 0.54) and median time to stop bleeding (91, 70, 77 seconds; p = 0.28). There was a statistical difference in median blood loss volume favoring SOFT-T (SAM XT, 686 mL; CAT, 624 mL; SOFT-T, 433 mL; p = 0.03). All 16 participants with previous experience were able to successfully place the tourniquet compared with 81 (62%) of 131 first-time users (p = 0.008). Conclusion No one should die of extremity hemorrhage, and civilians are our first line of defense. We demonstrate that when an untrained layperson is handed a commonly accepted tourniquet, failure is unacceptably high. Current devices are not intuitive and require training beyond the enclosed instructions. Plans to further evaluate this cohort after formal "Stop the Bleed" training are underway.

SummaryBackground Short-term medical missions (STMMs) refer to the provision of direct pro bono medical services in lower and middle income countries for periods ranging from days to a few weeks by physicians from rich countries. Survey data have provided limited information on demographic and professional profiles of physicians as well as monetary and manpower inputs. Understanding why physicians participate, however, remains incomplete. The study's objective was to elicit physicians' motivations directly. Methods Semi-structured interviews of physicians identified through snowball recruiting were conducted to explore motivational themes and then analyzed using narrative software employing directed content analysis methodology. Results Twenty physicians from varying backgrounds and specialties were interviewed. Responses identified aspects of the decision to participate and the relative influence of economic, diplomatic, value-related, and emotive constructs. Conclusion Personality traits may be more influential to participation than demographic, professional, or socioeconomic determinants. Word-of-mouth recruitment appears to underlie the increase in STMM activity, facilitated by information technology. Reported key motivators for physician participation in STMMs tend to parallel schools of thought regarding philanthropy and volunteering and include satisfaction from helping in challenging conditions of limited resources, learning experiences, appreciation from patients, sense of renewal, and the legacy effect of teaching.

Cognitive impairment is common in patients with pain. While symptoms of pain are effectively treated with osteopathic manipulative treatment (OMT), the cognitive complaint is vastly ignored. Pain-induced cognitive dysfunction can be severe and is particularly apparent in working memory and attention. There is good reason to expect cognitive responsiveness to OMT. Previous research has reported the effects of OMT on related psychiatric outcomes, including relief from depression and anxiety, suggesting that OMT may produce more cortical benefits than is currently thought. The rationale to link OMT to cognition comes from the tenets of osteopathic medicine: body unity, homeostasis, and the structure-function relationship. The present article provides background evidence to support the hypothetical link between OMT and cognitive benefits and proposes a physiological mechanism of how OMT could exert its effect on cognition. Research strategies are discussed to test the hypotheses that are generated from the proposed theoretical framework.

One of the greatest conundrums with tourniquet (TQ) education is the use of an appropriate surrogate of hemorrhage in the training setting to determine whether a TQ has been successfully used. At our facility, we currently use loss of audible Doppler signal or loss of palpable pulse to represent adequate occlusion of vasculature and thus successful TQ application. We set out to determine whether pain can be used to indicate successful TQ application in the training setting.Three tourniquet systems (a pneumatic tourniquet, Combat Application Tourniquet® [C-A-T], and Stretch Wrap and Tuck Tourniquet™ [SWAT-T]) were used to occlude the arterial vasculature of the left upper arm (LUA), right upper arm (RUA), left forearm (LFA), right forearm (RFA), right thigh (RTH), and right calf (RCA) of 41 volunteers. A 4MHz, handheld Doppler ultrasound was used to confirm loss of Doppler signal (LOS) at the radial or posterior tibial artery to denote successful TQ application. Once successful placement of the TQ was noted, subjects rated their pain from 0 to 10 on the visual analog scale. In addition, the circumference of each limb, the pressure with the pneumatic TQ, number of twists with the C-A-T, and length of TQ used for the SWAT-T to obtain LOS was recorded.All 41 subjects had measurements at all anatomic sites with the pneumatic TQ, except one participant who was unable to complete the LUA. In total, pain was rated as 1 or less by 61% of subjects for LUA, 50% for LFA, 57.5% for RUA, 52.5% RFA, 15% for RTH, and 25% for RCA. Pain was rated 3 or 4 by 45% of subjects for RTH. For the C-A-T, data were collected from 40 participants. In total, pain was rated as 1 or less by 57.5% for the LUA, 70% for the LFA, 62.5% for the RUA, 75% for the RFA, 15% for the RTH, and 40% for the RCA. Pain was rated 3 or 4 by 42.5%. The SWAT-T group consisted of 37 participants for all anatomic locations. In total, pain was rated as 1 or less by 27% for LUA, 40.5% for the LFA, 27.0% for the RUA, 43.2 for the RFA, 18.9% for the RTH, and 16.2% for the RCA. Pain was rated 5 by 21.6% for RTH application, and 3 or 4 by 35%.The unexpected low pain values recorded when loss of signal was reached make the use of pain too sensitive as an indicator to confirm adequate occlusion of vasculature and, thus, successful TQ application.

Introduction: Individuals with visible differences due to medical conditions, such as systemic sclerosis (SSc; scleroderma), have reported difficulty navigating social situations because of issues such as staring, invasive questions, and rude comments. Fears or anxiety linked to situations in which a person interacts with others is known as social interaction anxiety. However, there exists no validated measurement tool to examine social interaction anxiety in rheumatologic conditions. Methods: The present study examines the reliability (internal consistency) and validity (structural and convergent) of the Social Interaction Anxiety Scale-6 (SIAS-6) in a sample of 802 individuals with SSc, and compares these psychometric properties across limited and diffuse subtypes of the disease. Multi-group confirmatory factor analysis was used to examine the factor structure of the SIAS-6 in patients with both limited and diffuse SSc. Results: A one-factor structure was found to fit well for individuals with SSc with both limited and diffuse disease. The measure demonstrated strong internal consistency reliability and convergent validity with relevant measures in expected magnitudes and directions. Conclusions: The SIAS-6 is a psychometrically robust measure that can confidently be used in SSc populations to examine social interaction anxiety. Moreover, scores can meaningfully be compared between patients with limited and diffuse disease., MD Anderson Cancer Center - University of Texas MD Anderson Cancer Center(undefined), Financial support: The Scleroderma Patient-centered Intervention Network (SPIN) is funded by a Canadian Institutes of Health Research (CIHR) Emerging Team Grant for Rare Diseases (PI, Thombs; TR3-119192). In addition to CIHR funding, SPIN has received institutional contributions from the Lady Davis Institute for Medical Research of the Jewish General Hospital, Montréal, Canada and from McGill University, Montréal, Canada. SPIN has also received support from the Scleroderma Society of Ontario, Scleroderma Canada, and Sclérodermie Québec. Ms. Gholizadeh’s work on this project was supported by a Rheumatology Research Foundation: Health Professional Research Preceptorship. Dr. Kwakkenbos was supported by a CIHR Banting Postdoctoral Fellowship. Ms. Jewett was supported by a CIHR Doctoral Research Award. Dr. Thombs was supported by an Investigator Salary Award from the Arthritis Society

BACKGROUND: Short term medical missions (STMMs) are a form of unregulated and unsanctioned, grass roots, direct medical service aid from wealthier countries to low and middle income countries. The US leads the world in STMM activity. The magnitude of monetary and man power inputs towards STMMs is not clear. The objective of this study is to estimate the prevalence of physician participation in STMMs from the US and the related expenditures of cash and resources.METHODS: An online survey solicited information on physician participation in STMMs. Responses regarding costs were aggregated to estimate individual and global expenditures.RESULTS: Sample statistics from 601 respondent physicians indicate an increasing participation by US physicians in STMMs. Including opportunity cost, average total economic inputs for an individual physician pursuing an STMM exceed $11,000. Composite expenditures for STMM deployment from the US are estimated at near $3.7 billion annually and the resource investment equates with nearly 5800 physician fulltime equivalents.CONCLUSIONS: STMM participation and mission numbers have been increasing in the millennium. The aggregate costs are material when benchmarked against formal US aid transfers. Understanding the drivers of physician volunteerism in this activity is thereby worthy of study and relevant to future policy deliberation.

Item does not contain fulltext Body concealment is an important component of appearance distress for individuals with disfiguring conditions, including scleroderma. The objective was to replicate the validation study of the Body Concealment Scale for Scleroderma (BCSS) among 897 scleroderma patients. The factor structure of the BCSS was evaluated using confirmatory factor analysis and the Multiple-Indicator Multiple-Cause model examined differential item functioning of SWAP items for sex and age. Internal consistency reliability was assessed via Cronbach's alpha. Construct validity was assessed by comparing the BCSS with a measure of body image distress and measures of mental health and pain intensity. Results replicated the original validation study, where a bifactor model provided the best fit. The BCSS demonstrated strong internal consistency reliability and construct validity. Findings further support the BCSS as a valid measure of body concealment in scleroderma and provide new evidence that scores can be compared and combined across sexes and ages. 8 p.

Short-term medical missions (STMMs) refer to the provision of direct pro bono medical services in lower and middle income countries for periods ranging from days to a few weeks by physicians from rich countries. Survey data have provided limited information on demographic and professional profiles of physicians as well as monetary and manpower inputs. Understanding why physicians participate, however, remains incomplete. The study's objective was to elicit physicians' motivations directly.Semi-structured interviews of physicians identified through snowball recruiting were conducted to explore motivational themes and then analyzed using narrative software employing directed content analysis methodology.Twenty physicians from varying backgrounds and specialties were interviewed. Responses identified aspects of the decision to participate and the relative influence of economic, diplomatic, value-related, and emotive constructs.Personality traits may be more influential to participation than demographic, professional, or socioeconomic determinants. Word-of-mouth recruitment appears to underlie the increase in STMM activity, facilitated by information technology. Reported key motivators for physician participation in STMMs tend to parallel schools of thought regarding philanthropy and volunteering and include satisfaction from helping in challenging conditions of limited resources, learning experiences, appreciation from patients, sense of renewal, and the legacy effect of teaching.

Contains fulltext : 161778.PDF (Publisher’s version ) (Open Access) Background: Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood. Objectives: To gain an in-depth understanding of the emotional experiences and sources of emotional distress for women and men living with scleroderma through focus group discussions. Methods Three semi-structured focus group discussions were conducted (two in English, one in French) with a total of 22 people with scleroderma recruited through the Scleroderma Society of Ontario in Hamilton, Ontario and a scleroderma clinic in Montreal, Canada. Interviews were recorded, transcribed, and then coded for emerging themes using thematic inductive analysis. Results: Core themes representing sources of emotional distress were identified, including: (a) facing a new reality; (b) the daily struggle of living with scleroderma; (c) handling work, employment and general financial burden; (d) changing family roles; (e) social interactions; and (f) navigating the health care system. Collectively, these themes refer to the stressful journey of living with scleroderma including the obstacles faced and the emotional experiences beginning prior to receiving a diagnosis and continuing throughout the participants' lives. Conclusion: Scleroderma was portrayed as being an unpredictable and overwhelming disease, resulting in many individuals experiencing multiple sources of emotional distress. Interventions and supportive resources need to be developed to help individuals with scleroderma and people close to them manage and cope with the emotional aspects of the disease. 15 p.

Objectives. SSc-associated gastrointestinal tract involvement (SSc-GIT) is an important predictor of depressive symptoms. University of California at Los Angeles Scleroderma Clinical trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0) is a 34-item valid instrument that captures GIT symptom severity and impact on quality of life. It has seven GI-specific scales and a total GIT score. The objectives were to assess: (i) whether there is an association between depressed mood with GI symptom scales as assessed by the UCLA SCTC GIT 2.0 instrument; and (ii) to explore which GI-specific symptom scales are associated with depressed mood in patients with SSc. Methods. One hundred and fifty-two patients with SSc completed the UCLA SCTC GIT 2.0 and the Center for Epidemiologic Studies Short Depression scale (CES-D10). Patients were divided into depressed (CES-D5 10) or non-depressed group (CES-D < 10) and compared using t-test or chi-square test. Multiple linear regression was used to determine associations between GI scales and depressed mood (CES-D). Results. Study participants were 84% female, 78% Caucasian and 40% had depressed mood (CES-D105 10). Patients with depressed mood had statistically worse GI scale scores (except fecal soilage) and worse total GIT score (P < 0.05). In the multivariable model reflux and constipation scales were independently associated with worse CES-D scores (P = 0.010.06) Conclusion. SSc-GIT involvement is associated with depressed mood. Reflux and constipation scales of UCLA-SCTC GIT 2.0 were independently associated with CES-D. Future studies should assess if treatment of GIT symptoms will improve depressed mood in patients with SSc-GIT.

Systemic Sclerosis (Scleroderma, SSc) is a disease of unknown etiology characterized by widespread vasculopathy and extracellular matrix deposition leading to fibrosis and autoimmune processes. Digital ischemia (digital ulcers (DUs)) is the hallmark of SSc-related vasculopathy and is characterized by endothelial dysfunction leading to intimal proliferation and thrombosis. It happens frequently (30% of the patients each year) and it is associated with significant morbidity. This paper summarizes the current information regarding pathogenesis, definitions, management, and exploratory therapies in DUs associated with SSc.

Objective.Raynaud’s phenomenon (RP) is an important clinical feature of systemic sclerosis (SSc) for which consistently effective therapies are lacking. The study was designed to assess the safety, tolerability, and efficacy of tadalafil, a selective, long acting type V cyclic GMP phosphodiesterase (PDE-5) inhibitor, in this clinical syndrome.Methods.We performed a prospective, randomized, double-blind, placebo-controlled, crossover study comparing oral tadalafil at a fixed dose of 20 mg daily for a period of 4 weeks versus placebo in women with RP secondary to SSc.Results.Thirty-nine subjects completed the study and were evaluable. There were no statistically significant differences in Raynaud Condition Score (RCS), frequency of RP episodes, or duration of RP episodes between treatment groups. Placebo response was a confounding factor. Tadalafil was well tolerated.Conclusion.Tadalafil appears to be safe and well tolerated but lacks efficacy in comparison to placebo as a treatment for RP secondary to SSc.

Objective. To refine the previously developed scleroderma (systemic sclerosis [SSc]) gastrointestinal tract (GIT) instrument (SSC-GIT 1.0). Methods. We administered the SSC-GIT 1.0 and the Short Form 36 to 152 patients with SSc; 1 item was added to the SSC-GIT 1.0 to assess rectal incontinence. In addition, subjects completed a rating of the severity of their GIT involvement (from very mild to very severe). Evaluation of psychometric properties included internal consistency reliability, test– retest reliability (mean time interval 1.1 weeks), and multitrait scaling analysis. Results. Study participants were mostly women (84%) and white (81%); 55% had diffuse SSc. Self-rated severity of GIT involvement ranged from no symptoms to very mild (39%), mild (21%), moderate (31%), and severe/very severe (9%). Of an initial 53 items in the SSC-GIT 1.0, 19 items were excluded, leaving a 34-item revised instrument (the University of California, Los Angeles Scleroderma Clinical Trial Consortium GIT 2.0 [UCLA SCTC GIT 2.0]). Analyses supported 7 multi-item scales: reflux, distention/bloating, diarrhea, fecal soilage, constipation, emotional well-being, and social functioning. Test–retest reliability estimates were >0.68 and coefficient alphas were >0.67. Participants who rated their GIT disease as mild had lower scores on a 0 –3 scale on all 7 scales. Symptom scales were also able to discriminate subjects with corresponding clinical GIT diagnoses. The Total GIT Score, developed by averaging 6 of 7 scales (excluding constipation), was reliable and provided greater discrimination between mild, moderate, and severe self-rated GIT involvement than individual scales. Conclusion. This study provides support for the reliability and validity of the UCLA SCTC GIT 2.0, an improvement over the SSC-GIT 1.0, and supports a Total GIT Score in SSc patients with GIT.

America scored at the top of the World Giving Index survey in 2014. This paper reports on a survey among US physicians about preferences for and prevalence of pro-bono activities devoted to health-related direct medical services and medical teaching. Profiles related to these preferences are described. An online survey solicited information on physician participation in selected charitable and volunteer activities. The responses were descriptively tabulated and multivariate regressions were performed to identify associations between the activities and demographic and professional features. Six hundred one qualified respondents (0.62 % response rate) indicated participation in one or more pro-bono activities. Seventy-two percent performed activities domestically and 32 % provided services abroad in lower and middle income countries. Uncompensated medical teaching (54 %) was the most common activity followed by waiving fees in their practices (39 %). No clear profile emerged that predicts a subset of physicians likely to perform a particular activity. Unpaid teaching and direct pro-bono services reflect the medical profession’s contribution to American generosity. Amounts of fees waived in practice and engagement in volunteer activities have kept pace with historic benchmarks in support of unmet needs for these social goods. Participation in pro-bono activities appears broad and not the selective domain of particular physician subsets.

Objective: This study determined the prevalence of at-risk drinking in a psychiatric emergency service and compared the characteristics and functioning of at-risk drinkers with schizophrenia or bipolar disorder with those of atrisk drinkers with depression or anxiety disorders. Methods: Adult patients who entered the psychiatric emergency service and met study criteria were surveyed. Results: A total of 148 participants had schizophrenia or bipolar disorder, and 242 had depression or anxiety. Twenty-three percent of the group with schizophrenia or bipolar disorder and 22 percent of the group with depression or anxiety drank more than the recommended limits. The group with schizophrenia or bipolar disorder reported experiencing significantly more consequences from drinking than the depression or anxiety group. Both groups reported significant depression in the prior few days. Conclusions: This study demonstrated the importance of assessing alcohol use and depression among all patients in psychiatric emergency services. (Psychiatric Services 57: 1039–1042, 2006)

Introduction: Psychosocial and rehabilitation interventions are increasingly used to attenuate disability and improve health-related quality of life (HRQL) in chronic diseases, but are typically not available for patients with rare diseases. Conducting rigorous, adequately powered trials of these interventions for patients with rare diseases is difficult. The Scleroderma Patient-centered Intervention Network (SPIN) is an international collaboration of patient organisations, clinicians and researchers. The aim of SPIN is to develop a research infrastructure to test accessible, low-cost self-guided online interventions to reduce disability and improve HRQL for people living with the rare disease systemic sclerosis (SSc or scleroderma). Once tested, effective interventions will be made accessible through patient organisations partnering with SPIN. Methods and analysis: SPIN will employ the cohort multiple randomised controlled trial (cmRCT) design, in which patients consent to participate in a cohort for ongoing data collection. The aim is to recruit 1500– 2000 patients from centres across the world within a period of 5 years (2013–2018). Eligible participants are persons ≥18 years of age with a diagnosis of SSc. In addition to baseline medical data, participants will complete patient-reported outcome measures every 3 months. Upon enrolment in the cohort, patients will consent to be contacted in the future to participate in intervention research and to allow their data to be used for comparison purposes for interventions tested with other cohort participants. Once nterventions are developed, patients from the cohort will be randomly selected and offered interventions as part of pragmatic RCTs. Outcomes from patients offered interventions will be compared with outcomes from trial-eligible patients who are not offered the interventions. Ethics and dissemination: The use of the cmRCT design, the development of self-guided online interventions and partnerships with patient organisations will allow SPIN to develop, rigourously test and effectively disseminate psychosocial and rehabilitation interventions for people with SSc., (undefined)

The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) registry was designed to advance the understanding of the pathogenesis of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). This registry provides the opportunity to examine both microvascular and macrovascular complications in SSc. In this manuscript we review SSc-PAH therapies and report on their use in the PHAROS registry.

Objective.To provide minimally important difference (MID) estimates for the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0) in a longitudinal observational cohort.Methods.We administered the UCLA SCTC GIT 2.0 to 115 patients with systemic sclerosis (SSc) at 2 timepoints 6 months apart. The UCLA SCTC GIT 2.0 has 7 multi-item scales: Reflux, Distension/Bloating, Diarrhea, Fecal Soilage, Constipation, Emotional Well-being, and Social Functioning and a total GIT score. All scales are scored from 0 [better health-related quality of life (HRQOL)] to 3 (worse HRQOL) except the diarrhea and constipation scales (ranges 0–2 and 0–2.5, respectively). Patients also rated their overall and upper and lower GIT involvement during the second visit using a response scale with options “much better; somewhat better; almost the same; somewhat worse; or much worse.” The minimally changed group was defined by those reporting they were somewhat better or somewhat worse compared to first visit.Results.Study participants were 84% female and 81% white with a mean disease duration of 6.9 years. The MID estimates for improvement ranged from 0.07 for the Social Functioning scale to 0.36 for the Emotional Well-being scale. For worsening, the MID estimates ranged from 0.06 for the Fecal Soilage scale to 0.21 for the Social Functioning scale.Conclusion.We provide MID estimates for the UCLA SCTC GIT 2.0 scales. This information can aid in interpreting scale scores in future randomized controlled trials and observational studies.

Systemic sclerosis- (SSc-) related vasculopathy, as manifested by Raynaud's Phenomenon (RP) and digital ulcers (DUs), is associated with significant impairment of the quality of life and morbidity. The current vasoactive approach for SSc-RP, although employing vasodilators, is entirely off-label. PDE-5 inhibitors improve peripheral circulation, are well tolerated, and are widely used for various forms of constrictive vasculopathies. This class of medications has become one of the first lines of treatment of SSc-RP and SSc-DUs among rheumatologists that routinely treat SSc patients. Due to the lack of robust randomized clinical trials of PDE-5 inhibitors in SSc-RP/DUs, the PDE-5 inhibitors have not been FDA approved for these particular indications, which constitutes a significant barrier to prescribing this category of drugs. This paper reviews the current state of evidence-based knowledge in SSc-related vasculopathy and the use of PDE-5 inhibitors.

Objective.To describe the magnitude and correlates of discordance between patient and physician assessments of disease severity in patients with systemic sclerosis (SSc).Methods.Subjects were patients enrolled in the Canadian Scleroderma Research Group Registry. The outcomes of interest were patient and physician global assessments of disease severity (scales ranging from 0–10). Predictors of disease severity represented the spectrum of disease in SSc (skin involvement, severity of Raynaud’s phenomenon, shortness of breath, gastrointestinal symptoms and pain, number of fingertip ulcers, tender and swollen joints, creatinine, and fatigue). The results of the analysis were validated in an independent sample of patients with SSc from the United States.Results.Patients perceived greater disease severity than physicians (mean difference 0.78 ± 2.65). The agreement between patient and physician assessments of disease severity was, at best, modest (intraclass correlation 0.3774; weighted κ 0.3771). Although both patients and physicians were influenced by skin scores, breathlessness, and pain, the relative importance of these predictors differed. Patients were also influenced by other subjective symptoms, while physicians were also influenced by disease duration and creatinine. The predictors explained 56% of the deviance in the patient global assessments and 29% in the physician assessments. These findings were confirmed in the US dataset.Conclusion.Patients and physicians rate SSc disease severity differently in magnitude and are influenced by different factors. Patient-assessed and physician-assessed measures of severity should be considered as complementary and used together in future studies of SSc.

Sexual dysfunction is common in systemic sclerosis (SSc). Male erectile dysfunction (MED) has been reported in around 80% of subjects and more than half of female patients fulfill criteria for diagnosis as female sexual arousal Disorder (FSAD). While some evidence supports a role for cavernosal fibrosis, abundant data suggest that MED is yet another clinical feature of SSc related to vasculopathy. The contribution of vasculopathy to the more complex issues of female sexual dysfunction is less clear. Inhibitors of Type V phosphodiesterase are effective in men with MED secondary to SSc. Limited study in women suggests inconsistent effects on behavior (frequency) but not on measures related to perfusion. Sexual activity is an important component of quality of life and an important domain for the caregiver to address; it is not clear that it warrants primary consideration as a consistent measure of scleroderma-related vasculopathy.

The 6-min walk test (6MWT) is a standardized measure of submaximal exercise capacity that has served as the primary measure of outcome in studies of pulmonary arterial hypertension. Never fully validated in scleroderma, a variety of data suggest that many non-pulmonary aspects of scleroderma contribute to its results thereby blunting the ability of the 6MWT to measure change in lung function. Although reproducible, the lack of correlation with measures of parenchymal lung disease demonstrates an insurmountable lack of both construct and content validity. The 6MWT should be abandoned as an outcome measure in lung disease complicating scleroderma.

• Lung disease is the leading cause of mortality and late disease morbidity in systemic sclerosis • Pulmonary hypertension (PH) affects between 8 and 10% of patients with scleroderma and is associated with a poor prognosis • Scleroderma patients with combined interstitial lung disease (SILD) and PH are a distinct subpopulation often excluded or under-represented in clinical trials • Doppler echocardiography (DE) is commonly used to screen for PH in this population

Background Short-term medical missions (STMMs) represent a grass-roots form of aid, transferring medical services rather than funds or equipment. The objective of this paper is to review empirical studies on social, economic and diplomatic aspects of STMMs. Methods A systematic literature review was conducted by searching PubMed and EBSCOhost for articles published from 1947–2014 about medical missions to lower and middle income countries (LMICs). Publications focused on military, disaster and dental service trips were excluded. A data extraction process was used to identify publications relevant to our objective stated above. Results PubMed and EBSCOhost searches provided 4138 and 3262 articles respectively for review. Most articles that provide useful information have appeared in the current millennium and are found in focused surgical journals. Little attention is paid to aspects of volunteerism, altruism and philanthropy related to STMM activity in the literature reviewed (1 article). Evidence of professionalization remains scarce, although elements including guidelines and tactical instructions have been emerging (27 articles). Information on costs (10 articles) and commentary on the relevance of market forces (1 article) are limited. Analyses of spill-over effects, i.e., changing attitudes of physicians or their communities towards aid, and characterizations of STMMs as meaningful foreign aid or strategic diplomacy are few (4 articles). Conclusions The literature on key social, economic and diplomatic aspects of STMMs and their consequences is sparse. Guidelines, tactical instructions and attempts at outcome measures are emerging that may better professionalize the otherwise unregulated activity. A broader discussion of these key aspects may lead to improved accountability and intercultural professionalism to accompany medical professionalism in STMM activity. Electronic supplementary material The online version of this article (doi:10.1186/s12913-015-0980-3) contains supplementary material, which is available to authorized users.

Introduction: Systemic sclerosis (SSc) is characterized by fibrosis and microvascular abnormalities including dysregulated angiogenesis. Chemokines, in addition to their chemoattractant properties, have the ability to modulate angiogenesis. Chemokines lacking the enzyme-linked receptor (ELR) motif, such as monokine induced by interferon-g (IFN-g) (MIG/CXCL9) and IFN-inducible protein 10 (IP-10/CXCL10), inhibit angiogenesis by binding CXCR3. In addition, CXCL16 promotes angiogenesis by binding its unique receptor CXCR6. In this study, we determined the expression of these chemokines and receptors in SSc skin and serum. Methods: Immunohistology and enzyme-linked immunosorbent assays (ELISAs) were used to determine chemokine and chemokine receptor expression in the skin and serum, respectively, of SSc and normal patients. Endothelial cells (ECs) were isolated from SSc skin biopsies and chemokine and chemokine receptor expression was determined by quantitative PCR and immunofluorescence staining. Results: Antiangiogenic IP-10/CXCL10 and MIG/CXCL9 were elevated in SSc serum and highly expressed in SSc skin. However, CXCR3, the receptor for these chemokines, was decreased on ECs in SSc vs. normal skin. CXCL16 was elevated in SSc serum and increased in SSc patients with early disease, pulmonary arterial hypertension, and those that died during the 36 months of the study. In addition, its receptor CXCR6 was overexpressed on ECs in SSc skin. At the mRNA and protein levels, CXCR3 was decreased while CXCR6 was increased on SSc ECs vs. human microvascular endothelial cells (HMVECs). Conclusions: These results show that while the expression of MIG/CXCL9 and IP-10/CXCL10 are elevated in SSc serum, the expression of CXCR3 is downregulated on SSc dermal ECs. In contrast, CXCL16 and CXCR6 are elevated in SSc serum and on SSc dermal ECs, respectively. In all, these findings suggest angiogenic chemokine receptor expression is likely regulated in an effort to promote angiogenesis in SSc skin.