Pauling and Frech reply

We thank Dr. Hughes for his interest1 in our article2 reporting factors influencing Raynaud phenomenon (RP) symptom reporting in patients with systemic sclerosis (SSc). We presented data demonstrating differences in Raynaud symptom reporting using the Raynaud Condition Score (RCS) diary depending on season of enrollment2.</p

Pesticides As Pollutants Under The Clean Water Act

Climate change and shifting weather patterns affect the development and distribution of insects and other pests. Public agencies as well as private parties, such as farmers and ranchers, commonly use chemical and biological pesticides to combat the spread and entrenchment of these nuisances. Given the ubiquitous use of pesticides, it is not surprising that pesticide regulation involves important issues of law and public policy

Factors influencing Raynaud condition score diary outcomes in systemic sclerosis

Objective. Raynaud phenomenon (RP) in systemic sclerosis (SSc) could be influenced by clinical phenotype, environmental factors (e.g., season), and personal factors (e.g., coping strategies and ill-health perceptions). We studied the relative influence of a range of putative factors affecting patient-reported assessment of SSc-RP severity. Methods. SSc patients were enrolled at UK and US sites. Participants completed the 2-week Raynaud Condition Score (RCS) diary alongside collection of patient demographics, clinical phenotype, the Coping Strategies Questionnaire, Pain Catastrophizing Scale, Scleroderma Health Assessment Questionnaire (SHAQ), and both patient/physician visual analog scale (VAS) assessments for RP, digital ulcer disease, and global disease. Environmental temperature data were obtained at each site. A second RCS diary was completed 6 months after enrollment. Results. We enrolled 107 patients (baseline questionnaires returned by 94). There were significant associations between RCS diary variables and both catastrophizing and coping strategies. There were significant associations between RCS diary outcomes and both environmental temperature and season of enrollment. Age, disease duration, sex, disease subtype, smoking, and vasodilator use were not associated with RCS diary outcomes. The best-fitting multivariate model identified the patient RP VAS, SHAQ pain VAS, and SHAQ gastrointestinal VAS subscales as the strongest independent predictors of the RCS. Conclusion. Patient-reported assessment of SSc-RP severity is associated with a number of factors including pain, catastrophizing, and coping strategies. The effects of seasonal variation in environmental temperature on SSc-RP burden has implications for clinical trial design. Treatments targeting SSc-RP pain and the development of behavioral interventions enhancing coping strategies may reduce the burden of SSc-RP.</p

The Prevalence and Clinical Correlates of an Auscultatory Gap in Systemic Sclerosis Patients

Introduction. Accurate blood pressure (BP) measurement is essential to the diagnosis and management of hypertension in patients with systemic sclerosis (SSc) to help prevent renal and cardiovascular complications. The presence of an auscultatory gap during manual BP measurement—the temporary disappearance of the Korotkoff sounds during cuff deflation—leads to a potentially important underestimate of systolic BP if undetected. Objectives. Since the presence of an auscultatory gap is frequently associated with increased vascular stiffness, we investigated its presence and correlates in 50 consecutive SSc patients. Methods. For each patient, BP was measured sequentially using three different approaches performed in the same order. Results. Sixteen of 50 patients (32%) had an auscultatory gap which if undetected would have resulted in clinically important underestimates of systolic BP in 4 patients. The presence of an auscultatory gap was statistically associated with the presence of antibodies to RNA polymerase III (P<0.0068) and SSc diagnosis type (P<0.01). Conclusions. Our study demonstrates that auscultatory gaps are relatively common in SSc and correlate with markers for SSc vasculopathy. If undetected auscultatory gaps may result in clinically important underestimation of BP. Thus, electronic oscillometric BP may be preferred in SSc patients

Effect of coping strategies on patient and physician perceptions of disease severity and disability in systemic sclerosis

Objective. Systemic sclerosis (SSc) results in impaired function, disability, and reduced health-related quality of life. We investigated the effect of coping strategies on the patient global assessment of health (PtGA) and Health Assessment Questionnaire–Disability Index (HAQ-DI), after controlling for clinical characteristics and disease activity. We also explored the relationship between coping strategies and the correlation between the PtGA and physician global assessment (PGA) in SSc. Methods. We undertook posthoc analyses using baseline data obtained from the Raynaud Symptom Study (RSS). The PtGA, Coping Strategies Questionnaire, Pain Catastrophizing Scale, and Scleroderma Health Assessment Questionnaire were collected alongside the PGA, clinical characteristics, and patient demographics. Multivariable linear regression models and correlations were used to evaluate the relationship between coping strategies with the PtGA, HAQ-DI, and PGA. Results. Of the 107 patients with SSc enrolled in the RSS, there were sufficient data available for the analysis of 91 participants. The mean PtGA was 40/100 (SD 27) and the mean HAQ-DI was 0.87/3.0 (SD 0.73). After controlling for clinical and patient demographics, pain catastrophizing and maladaptive coping skills were significantly associated with the PtGA and HAQ-DI scores (P &lt; 0.05 for both), but not the PGA. Conclusion. The effect of coping strategies on PtGA and HAQ-DI (but not PGA in SSc) could influence the result of composite measures incorporating these outcome measures. Interventions to improve patient coping skills may support increased resilience and improve patient-perceived functional status and PtGA in SSc.</p

Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient-Reported Assessments of Disease Severity

Objective: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and the evolution of SSc-RP symptoms with disease progression. Methods: A cross-sectional study at UK and US sites captured digital color changes of SSc-RP and patients’ ability to identify with diagrammatic representations (and descriptive stems) of 4 distinct theoretical SSc-RP patterns (progressing severity A through D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, the clinical phenotype, the Scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary, and patient and physician global assessments were collected. Results: We enrolled 107 patients with SSc (with questionnaires returned by 94). A higher number of self-reported digital color changes of SSc-RP were associated with increased SSc-RP symptom severity but not with the SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity, and conceptually linked physician and patient assessments of peripheral vascular severity (e.g., SHAQ RP subscale and RCS diary parameters), but not with conceptually unrelated outcomes (e.g., SHAQ breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course. Conclusion: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischemia in patients with advanced SSc could influence diary-based approaches to assessing SSc-RP, with implications for future clinical trials.</p

Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report

Introduction Diffuse systemic sclerosis is associated with high mortality; however, the pathogenesis of cardiac death in these patients is not clear. Case presentation A 56-year-old Caucasian female patient presented with dyspnea and requested to donate her body to science in order to improve understanding of diffuse systemic sclerosis pathogenesis. She had extensive testing for dyspnea including pulmonary function tests, an echocardiogram, cardiac magnetic resonance imaging, and right heart catheterization to characterize her condition. Her case highlights the morbidity seen in this disease, including the presence of extensive skin thickening, digital ulcerations, and scleroderma renal crisis. Conclusion In this case report, we present the finding of cardiac tissue metabolomics, which may indicate a problem with vasodilation as a contributor to cardiac death in diffuse systemic sclerosis. The use of autopsy and tissue metabolomics in rare disease may help clarify disease pathogenesis

Valuation of scleroderma and psoriatic arthritis health states by the general public

<p>Abstract</p> <p>Objective</p> <p>Psoriatic arthritis (PsA) and scleroderma (SSc) are chronic rheumatic disorders with detrimental effects on health-related quality of life. Our objective was to assess health values (utilities) from the general public for health states common to people with PsA and SSc for economic evaluations.</p> <p>Methods</p> <p>Adult subjects from the general population in a Midwestern city (N = 218) completed the SF-12 Health Survey and computer-assisted 0-100 rating scale (RS), time trade-off (TTO, range: 0.0-1.0) and standard gamble (SG, range: 0.0-1.0) utility assessments for several hypothetical PsA and SSc health states.</p> <p>Results</p> <p>Subjects included 135 (62%) females, 143 (66%) Caucasians, and 62 (28%) African-Americans. The mean (SD) scores for the SF-12 Physical Component Summary scale were 52.9 (8.3) and for the SF-12 Mental Component Summary scale were 49.0 (9.1), close to population norms. The mean RS, TTO, and SG scores for PsA health states varied with severity, ranging from 20.2 to 63.7 (14.4-20.3) for the RS 0.29 to 0.78 (0.24-0.31) for the TTO, and 0.48 to 0.82 (0.24-0.34) for the SG. The mean RS, TTO, and SG scores for SSc health states were 25.3-69.7 (15.2-16.3) for the RS, 0.36-0.80 (0.25-0.31) for the TTO, and 0.50-0.81 (0.26-0.32) for the SG, depending on disease severity.</p> <p>Conclusion</p> <p>Health utilities for PsA and SSc health states as assessed from the general public reflect the severity of the diseases. These descriptive findings could have implications regarding comparative effectiveness research for tests and treatments for PsA and SSc.</p