Scleroderma Patient-centered Intervention Network Cohort Research Articles

Factors associated with physical function among people with systemic sclerosis: a SPIN cohort cross-sectional study.

To compare physical function in systemic sclerosis (SSc, scleroderma) to general population normative data and identify associated factors. Scleroderma Patient-centered Intervention Network Cohort participants completed the Physical Function domain of the Patient-Reported Outcomes Measurement Information System Version 2 upon enrolment. Multivariable linear regression was used to assess associations of sociodemographic, lifestyle, and disease-related variables. Among 2,385 participants, mean physical function T-score (43.7, SD = 8.9) was ∼2/3 of a standard deviation (SD) below the US general population (mean = 50, SD = 10). Factors associated in multivariable analysis included older age (-0.74 points per SD years, 95% CI -0.78 to -1.08), female sex (-1.35, -2.37 to -0.34), fewer years of education (-0.41 points per SD in years, -0.75 to -0.07), being single, divorced, or widowed (-0.76, -1.48 to -0.03), smoking (-3.14, -4.42 to -1.85), alcohol consumption (0.79 points per SD drinks per week, 0.45-1.14), BMI (-1.41 points per SD, -1.75 to -1.07), diffuse subtype (-1.43, -2.23 to -0.62), gastrointestinal involvement (-2.58, -3.53 to -1.62), digital ulcers (-1.96, -2.94 to -0.98), moderate (-1.94, -2.94 to -0.93) and severe (-1.76, -3.24 to -0.28) small joint contractures, moderate (-2.10, -3.44 to -0.76) and severe (-2.54, -4.64 to -0.44) large joint contractures, interstitial lung disease (-1.52, -2.27 to -0.77), pulmonary arterial hypertension (-3.72, -4.91 to -2.52), rheumatoid arthritis (-2.10, -3.64 to -0.56) and idiopathic inflammatory myositis (-2.10, -3.63 to -0.56). Physical function is impaired for many individuals with SSc and associated with multiple disease factors.

Factors associated with satisfaction with social roles and activities among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study

ObjectiveThe objectives were to (1) compare satisfaction with social roles and activities in a large multinational systemic sclerosis (SSc) cohort to general population normative data and (2) identify sociodemographic, lifestyle...

Patterns of patient-reported symptoms and association with sociodemographic and systemic sclerosis disease characteristics: a scleroderma Patient-centered Intervention Network (SPIN) Cohort cross-sectional study

Systemic sclerosis is a heterogenous disease in which little is known about patterns of patient-reported symptom clusters. We aimed to identify classes of individuals with similar anxiety, depression, fatigue, sleep disturbance, and pain symptoms and to evaluate associated sociodemographic and disease-related characteristics. This multi-centre cross-sectional study used baseline data from Scleroderma Patient-centered Intervention Network Cohort participants enrolled from 2014 to 2020. Eligible participants completed the PROMIS-29 v2.0 measure. Latent profile analysis was used to identify homogeneous classes of participants based on patterns of anxiety, depression, fatigue, sleep disturbance, and pain scores. Sociodemographic and disease-related characteristics were compared across classes. Among 2212 participants, we identified five classes, including four classes with "Low" (565 participants, 26%), "Normal" (651 participants, 29%), "High" (569 participants, 26%), or "Very High" (193 participants, 9%) symptom levels across all symptoms. Participants in a fifth class, "High Fatigue/Sleep/Pain and Low Anxiety/Depression" (234 participants, 11%) had similar levels of fatigue, sleep disturbance, and pain as in the "High" class but low anxiety and depression symptoms. There were significant and substantive trends in sociodemographic characteristics (age, education, race or ethnicity, marital or partner status) and increasing disease severity (diffuse disease, tendon friction rubs, joint contractures, gastrointestinal symptoms) across severity-based classes. Disease severity and sociodemographic characteristics of "High Fatigue/Sleep/Pain and Low Anxiety/Depression" class participants were similar to the "High" severity class. Most people with systemic sclerosis can be classified by levels of patient-reported symptoms, which are consistent across symptoms and highly associated with sociodemographic and disease-related variables, except for one group which reports low mental health symptoms despite high levels of other symptoms and substantial disease burden. Studies are needed to better understand resilience in systemic sclerosis and to identify and facilitate implementation of cognitive and behavioural strategies to improve coping and overall quality of life. National Institute of Nursing Research (F31NR019007), Canadian Institutes of Health Research, Arthritis Society Canada, the Lady Davis Institute for Medical Research, the Jewish General Hospital Foundation, McGill University, Scleroderma Society of Ontario, Scleroderma Canada, Sclérodermie Québec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, and Scleroderma Queensland.

Validity, Reliability, and Differential Item Functioning of English and French Versions of the 10-Item Connor-Davidson Resilience Scale in Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Some individuals with systemic sclerosis (SSc) report positive mental health, despite severe disease manifestations, which may be associated with resilience, but no resilience measure has been validated in SSc. This study was undertaken to assess the validity, reliability, and differential item functioning (DIF) between English- and French-language versions of the 10-item Connor-Davidson Resilience Scale (CD-RISC-10) in SSc. Eligible participants were enrolled in the Scleroderma Patient-centered Intervention Network Cohort and completed the CD-RISC-10 between August 2022 and January 2023. We used confirmatory factor analysis (CFA) to evaluate the CD-RISC-10 factor structure and conducted DIF analysis across languages with Multiple Indicators Multiple Causes models. We tested convergent validity with another measure of resilience and measures of self-esteem and depression and anxiety symptoms. We assessed internal consistency and test-retest reliability using Cronbach's alpha and intraclass correlation coefficient (ICC). A total of 962 participants were included in this analysis. CFA supported a single-factor structure (Tucker-Lewis index=0.99, comparative fit index=0.99, root mean square error of approximation=0.08 [90% confidence interval (90% CI) 0.07, 0.09]). We found no meaningful DIF. Internal consistency was high (α = 0.93 [95% CI 0.92, 0.94]), and we found that correlations with other measures of psychological functioning were moderate to large (|r| = 0.57-0.78) and confirmed study hypotheses. The scale showed good 1-2-week test-retest reliability (ICC 0.80 [95% CI 0.75, 0.85]) in a subsample of 230 participants. The CD-RISC-10 is a valid and reliable measure of resilience in SSc, with score comparability across English and French versions.

OP0277-HPR PATTERNS OF PATIENT-REPORTED SYMPTOMS IN SYSTEMIC SCLEROSIS: A SCLERODERMA PATIENT-CENTERED INTERVENTION NETWORK (SPIN) COHORT STUDY

BackgroundPatient-reported symptoms including pain, fatigue, sleep disturbance, anxiety, and depression are among the most common and burdensome symptoms in systemic sclerosis.[1–4] Most research on these symptoms in systemic sclerosis has examined only one symptom at a time, without evaluating the degree to which symptoms co-occur or how sociodemographic and disease-related characteristics are associated with co-occurring symptoms.ObjectivesWe aimed to identify homogenous subgroups, or classes, of individuals with systemic sclerosis who reported similar patterns of anxiety, depression, fatigue, sleep disturbance, and pain symptoms and to evaluate associated sociodemographic and disease-related characteristics.MethodsWe conducted a multi-center cross-sectional study using data collected at time of enrollment in the ongoing Scleroderma Patient-centered Intervention Network Cohort. Eligible participants completed the Patient-Reported Outcomes Measurement Information System (PROMIS)-29 profile. Latent profile analysis was used to identify classes of participants based on patterns of anxiety, depression, fatigue, sleep disturbance, and pain scores. Sociodemographic and disease-related characteristics were compared across classes.ResultsWe identified five distinct classes among 2,212 individuals with systemic sclerosis. Four of the classes were based on increasing levels of symptom severity across all symptoms, from “Low” (n=565, 26%) to “Normal” (n=651, 29%) to “High” (n=569, 26%) to “Very High” (n=193, 9%). Participants in the fifth class, labeled “High Fatigue/Sleep/Pain and Low Anxiety/Depression” (n=234, 11%), had similar levels of fatigue, sleep disturbance, and pain as in the “High” class but low symptoms of anxiety and depression. There were significant trends in sociodemographic characteristics (age, education, race or ethnicity, marital or partner status) and increasing disease severity (diffuse disease, tendon friction rubs, joint contractures, gastrointestinal symptoms) across severity-based groups. Disease severity and sociodemographic characteristics were similar between the “High Fatigue/Sleep/Pain and Low Anxiety/Depression” class and the “High” severity class.ConclusionThis is the first study to fully capture the co-occurrence of the most common symptoms in systemic sclerosis and to identify a group of people who appear to have avoided negative mental health outcomes that, for many patients, track closely with their disease severity and symptom levels. Studies are needed to better understand the characteristics of people with systemic sclerosis who are highly resilient and facilitate the implementation of multi-symptom interventions to better manage patient-reported symptoms and improve overall quality of life.

Patient Acceptable Symptom State for Burden From Appearance Changes in People With Systemic Sclerosis: A Cross-sectional Survey.

People with systemic sclerosis (SSc) often report substantial burden from appearance changes. We aimed to estimate the patient acceptable symptom state (PASS) for burden from appearance changes in people with SSc. We conducted a secondary analysis of the SCISCIF II study, a cross-sectional survey of 113 patients with SSc from France enrolled in the Scleroderma Patient-centered Intervention Network Cohort. Burden from appearance changes was assessed with a self-administered numeric rating scale (0, no burden to 10, maximal burden). Acceptability of the symptom state was assessed with a specific anchoring question. Participants who answered yes were in the group of patients who considered their symptom state as acceptable. The PASS for the burden from appearance changes was estimated with the 75th percentile method. Assessments of burden from appearance changes and answers to the anchoring question were available in 82/113 (73%) participants from the SCISCIF II study. Median age was 55 (IQR 24) years, mean disease duration 9.6 (SD 6.5) years and 32/80 (40%) participants had diffuse cutaneous SSc. The PASS estimate for the burden from appearance changes was 4.8 (95% CI 1.0-7.0) of 10 points. Our study provides a PASS estimate for burden from appearance changes. Our estimate could serve as a binary response criterion to assess the efficacy of treatments targeting burden from appearance changes.

Mental Health Care Use and Associated Factors in Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.

ObjectiveSystemic sclerosis (SSc) has significant psychosocial implications. We aimed to evaluate the proportion of participants in a large international SSc cohort who used mental health services in a 3‐month period and to evaluate demographic, psychological, and disease‐specific factors associated with use.MethodsBaseline data of participants enrolled in the Scleroderma Patient‐Centered Intervention Network Cohort were analyzed. We determined the proportion that used mental health services and the source of services in the 3 months prior to enrollment. Multivariable logistic regression was used to identify variables associated with service use.ResultsOf the 2319 participants included in the analysis, 417 (18%) used mental health services in the 3 months prior to enrollment. General practitioners were the most common mental health service providers (59%), followed by psychologists (25%) and psychiatrists (19%). In multivariable analysis, mental health service use was independently associated with higher education (odds ratio [OR] 1.07, 95% confidence interval [CI] 1.03‐1.11), smoking (OR 1.06, 95% CI 1.02‐1.11), being retired (OR 0.60, 95% CI 0.38‐0.93), having limited SSc (OR 1.39, 95% CI 1.02‐1.89), and having higher anxiety symptom scores (OR 1.04, 95% CI 1.03‐1.06) and lower self‐efficacy scores (OR 0.90, 95% CI 0.83‐0.97). Variables not significantly associated included age, race, disease manifestations, depression symptom scores, and body image distress.ConclusionAbout 18% of participants in a large international cohort received mental health services in a 3‐month period, of whom the majority received these services from a general practitioner.

Mental health before and during the pandemic in people with systemic sclerosis.

Mental health before and during the pandemic in people with systemic sclerosis.

Pain levels and associated factors in the Scleroderma Patient-centered Intervention Network (SPIN) cohort: a multicentre cross-sectional study.

Pain is an important and detrimental feature of systemic sclerosis but is often overlooked or deprioritised in research and clinical care. Raynaud's phenomenon, arthritis, and cutaneous ulcers are among the commonly reported disease manifestations of systemic sclerosis that could be associated with pain. We aimed to assess levels of pain intensity and interference and to evaluate disease factors associated with pain intensity and interference. In this multicentre cross-sectional study, participants from the Scleroderma Patient-centered Intervention Network cohort who completed pain intensity and interference measures (Patient Reported Outcomes Information System-29 profile, version 2·0) as part of baseline assessments were included. Patients were recruited from 46 centres in Australia, Canada, France, Mexico, Spain, the UK, and the USA between April 15, 2014, and Jan 7, 2020. Eligible patients included those aged 18 years or older who met the criteria for systemic sclerosis devised by the American College of Rheumatology and the European League Against Rheumatism. Associations of pain intensity and pain interference with systemic sclerosis-related variables and overlap syndromes, controlling for sociodemographic variables, were assessed with multiple linear regression. Continuous independent variables were standardised. Among 2157 participants with systemic sclerosis (268 [12%] males and 1889 [88%] females), 1870 (87%) reported mild, moderate, or severe pain (defined as ≥1 on a 0 to 10 scale), and 815 (38%) reported moderate or severe pain (defined as ≥5). Moreover, 757 (35%) participants reported moderate or severe pain interference. Greater pain intensity was independently associated with female sex (0·58 points [95% CI 0·26-0·90]), non-White race or ethnicity (0·50 points [0·21-0·79]), fewer years in formal education (0·30 points per SD [0·19-0·41]), country (reference: USA; Canada: 0·29 points [0·01-0·57] and UK: 0·58 points [0·21-0·95]), greater body-mass index (0·35 points per SD [0·24-0·45]); joint contractures (0·67 points [0·39-0·94]), digital ulcers (0·33 points [0·10-0·55]), gastrointestinal involvement (0·66 points [0·33-0·98]), skin involvement (measured using modified Rodnan skin score; 0·22 points per SD [0·10-0·35]), rheumatoid arthritis (0·96 points [0·50-1·43]), and Sjögren's syndrome (0·42 points [0·01-0·83]). Pain interference results were similar. Pain is common among people with systemic sclerosis. Controlling for sociodemographic variables, greater pain was associated with multiple systemic sclerosis-related manifestations, including joint contractures, digital ulcers, gastrointestinal involvement, skin involvement, and the presence of overlap syndromes. Health-care providers should work with patients to address pain, including identifying and addressing systemic sclerosis manifestations associated with their pain, and supporting behavioural approaches to minimise impact on function and quality of life. Canadian Institutes of Health Research, Arthritis Society, The Lady Davis Institute for Medical Research of the Jewish General Hospital, Jewish General Hospital Foundation, McGill University, Scleroderma Society of Ontario, Scleroderma Canada, Sclérodermie Québec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, and Scleroderma Queensland.

Pain and Self-Efficacy Among Patients With Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Pain is one of the most common symptoms affecting patients with systemic sclerosis; however, little is known about the relationship between self-efficacy and pain and changes in pain over time. The purpose of this study was to describe the relationships between self-efficacy and pain in patients with systemic sclerosis, as well as determine whether changes in self-efficacy mediate changes in pain. A prospective longitudinal study was conducted using data from the Scleroderma Patient-Centered Intervention Network Cohort. The baseline sample included 1,903 adults, with a trajectory subsample of 427 who completed 3-month assessments across 3 years. Hierarchical (sequential) forward multivariable regression, covarying for participant characteristics, was conducted to determine the association between self-efficacy and patient characteristics on pain outcomes. Trajectory models, covarying for participant characteristics, were used to examine changes in self-efficacy and pain outcomes across time and whether self-efficacy mediated the pain trajectories. Mean time since diagnosis was 9.5 years, with 39.2% diagnosed with diffuse cutaneous systemic sclerosis. Greater self-efficacy was associated with less pain interference and intensity. Increasing age, female gender, finger ulcers, and small joint contractures were related to greater pain interference and intensity. Esophageal gastrointestinal symptoms were associated with more pain interference. Self-efficacy and pain trajectories remained stable across time, and self-efficacy did not mediate the pain trajectories. This study identified self-efficacy, age, gender, finger ulcers, small joint contractures, and esophageal gastrointestinal symptoms as important correlates associated with pain in patients with systemic sclerosis. In addition, this study found that self-efficacy and pain outcomes remained stable over time, providing important insights into the longitudinal pain experiences of patients with systemic sclerosis.

Changes in mental health symptoms from pre-COVID-19 to COVID-19 among participants with systemic sclerosis from four countries: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort study

IntroductionNo studies have reported mental health symptom comparisons prior to and during COVID-19 in vulnerable medical populations. ObjectiveTo compare anxiety and depression symptoms among people with a pre-existing medical condition and factors associated with changes. MethodsPre-COVID-19 Scleroderma Patient-centered Intervention Network Cohort data were linked to COVID-19 data from April 2020. Multiple linear and logistic regression were used to assess factors associated with continuous change and ≥ 1 minimal clinically important difference (MCID) change for anxiety (PROMIS Anxiety 4a v1.0; MCID = 4.0) and depression (Patient Health Questionnaire-8; MCID = 3.0) symptoms, controlling for pre-COVID-19 levels. ResultsMean anxiety symptoms increased 4.9 points (95% confidence interval [CI] 4.0 to 5.7). Depression symptom change was negligible (0.3 points; 95% CI -0.7 to 0.2). Compared to France (N = 159), adjusted anxiety symptom change scores were significantly higher in the United Kingdom (N = 50; 3.3 points, 95% CI 0.9 to 5.6), United States (N = 128; 2.5 points, 95% CI 0.7 to 4.2), and Canada (N = 98; 1.9 points, 95% CI 0.1 to 3.8). Odds of ≥1 MCID increase were 2.6 for the United Kingdom (95% CI 1.2 to 5.7) but not significant for the United States (1.6, 95% CI 0.9 to 2.9) or Canada (1.4, 95% CI 0.7 to 2.5). Older age and adequate financial resources were associated with less continuous anxiety increase. Employment and shorter time since diagnosis were associated with lower odds of a ≥ 1 MCID increase. ConclusionsAnxiety symptoms, but not depression symptoms, increased dramatically during COVID-19 among people with a pre-existing medical condition.

Development of a new patient-reported outcome measure to assess activities and participation in people with systemic sclerosis: the Cochin 17-item Scleroderma Functional scale.

Patient-reported outcome measures (PROMs) aimed at assessing people with systemic sclerosis (SSc) have rarely involved the target population in the item- and domain-generation stage of the instrument construction. To develop a new PROM assessing activities and participation in people with SSc. A provisional International Classification of Functioning, Disability and Health (ICF)-based 65-item questionnaire previously developed from interviews of people with SSc was sent by email to all patients followed in the internal medicine department of Cochin hospital (n = 184) and enrolled in the Scleroderma Patient-centered Intervention Network Cohort. Items were reduced according to their metric properties. Dimensional structure of the questionnaire was assessed by principal component analysis, convergent and divergent validities by Spearman's rank correlation coefficient, internal consistency by Cronbach's α, and reliability by a test-retest method using the intraclass correlation coefficient (ICC) and Bland-Altman analysis. Overall, 113 of 184 patients (61·4%) completed the provisional questionnaire. The item-reduction process resulted in a 17-item questionnaire, the Cochin 17-item Scleroderma Functional scale (CSF-17). Principal component analysis extracted two dimensions: 10 items related to mobility (CSF-17 section A) and seven items related to general tasks (CSF-17 section B). We observed convergent validity of the CSF-17 total score with global activity limitation, pain, depression and aesthetic burden, and divergent validity with anxiety. Cronbach's α was 0·94 for section A and 0·95 for section B. ICC (n = 25 patients) was 0·92 for the CSF-17 total score. Bland-Altman analysis did not reveal a systematic trend for the test-retest. The CSF-17 is a new PROM assessing activities and participation specifically in people with SSc. Its content and construct validities are very high. What is already known about this topic? In the earliest stages of construction patient-reported outcomes (PROMs) for people with systemic sclerosis (SSc) rarely involve the target population. Instruments able to capture the specific needs of people with SSc in terms of activities and participation are lacking. What does this study add? The Cochin 17-item Scleroderma Functional Scale (CSF-17) is a new PROM assessing global activities and participation specifically in people with SSc. Patients' perspectives were prioritized at all stages of construction. What are the clinical implications of this work? The CSF-17 could be used in clinical practice and research to assess the efficacy of complex multidisciplinary interventions targeting activity limitations and participation restriction in people with SSc. Linked Comment: Clark and Denton. Br J Dermatol 2020; 183:610.

The Scleroderma Research Topics Survey for patients and health care professionals: A Scleroderma Patient-centered Intervention Network Project.

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.

Shortening patient-reported outcome measures through optimal test assembly: application to the Social Appearance Anxiety Scale in the Scleroderma Patient-centered Intervention Network Cohort

ObjectivesThe Social Appearance Anxiety Scale (SAAS) is a 16-item measure that assesses social anxiety in situations where appearance is evaluated. The objective was to use optimal test assembly (OTA) methods...

Reliability and Validity of Three Versions of the Brief Fear of Negative Evaluation Scale in Patients With Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Fear of negative evaluation is a common concern among individuals with visible differences but has received limited attention in systemic sclerosis (SSc), which can involve substantial changes to appearance. The Brief Fear of Negative Evaluation Scale (BFNE) was specifically designed to evaluate fear of negative evaluation. There are currently 3 versions of the BFNE with strong demonstrated measurement properties: two 8-item versions (BFNE-S, BFNE-8) and one 12-item version (BFNE-II). The present study evaluated these versions in SSc, and identified the most appropriate version for use among SSc patients. Participants were 1,010 patients with SSc enrolled in the Scleroderma Patient-Centered Intervention Network cohort. Multiple group confirmatory factor analysis, Cronbach's alpha, and Pearson's product-moment correlations were used to evaluate structural validity, internal consistency reliability, and convergent and divergent validity, respectively. Confirmatory factor analysis demonstrated that 1-factor models fit acceptably well for the 12-item BFNE-II, the 8-item BFNE-S, and the 8-item BFNE-8. Additionally, all Cronbach's alphas demonstrated excellent internal consistency reliability (BFNE-II = 0.98, BFNE-S = 0.97, BFNE-8 = 0.96), and all versions had comparable associations with measures of social anxiety, body-related attitudes, depression, age, and education. Psychometric support was found for all 3 versions of the BFNE, although the longer 12-item BFNE-II did not improve measurement compared to the shorter 8-item versions (BFNE-S and BFNE-8). Of these 2, the BFNE-S has been more widely studied, with strong validity data in a greater number of populations. Therefore, the BFNE-S is recommended to assess fear of negative evaluation among patients with SSc.

Validation of the Social Appearance Anxiety Scale in Patients With Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Systemic sclerosis (SSc) is an autoimmune disease that can cause disfiguring changes in appearance. This study examined the structural validity, internal consistency reliability, convergent validity, and measurement equivalence of the Social Appearance Anxiety Scale (SAAS) across SSc disease subtypes. Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort completed the SAAS and measures of appearance-related concerns and psychological distress. Confirmatory factor analysis (CFA) was used to examine the structural validity of the SAAS. Multiple-group CFA was used to determine whether SAAS scores can be compared across patients with limited and diffuse disease subtypes. Cronbach's alpha was used to examine internal consistency reliability. Correlations of SAAS scores with measures of body image dissatisfaction, fear of negative evaluation, social anxiety, and depression were used to examine convergent validity. SAAS scores were hypothesized to be positively associated with all convergent validity measures, with correlations significant and moderate to large in size. A total of 938 patients with SSc were included. CFA supported a 1-factor structure (Comparative Fit Index 0.92, Standardized Root Mean Residual 0.04, and Root Mean Square Error of Approximation 0.08), and multiple-group CFA indicated that the scalar invariance model best fit the data. Internal consistency reliability was good in the total sample (α = 0.96) and in disease subgroups. Overall, evidence of convergent validity was found with measures of body image dissatisfaction, fear of negative evaluation, social anxiety, and depression. The SAAS can be reliably and validly used to assess fear of appearance evaluation in patients with SSc, and SAAS scores can be meaningfully compared across disease subtypes.

Validation of the Social Interaction Anxiety Scale in scleroderma: a Scleroderma Patient-centered Intervention Network Cohort study.

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. 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. 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. 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.

Performance of the Patient-Reported Outcomes Measurement Information System-29 in scleroderma: a Scleroderma Patient-centered Intervention Network Cohort Study.

The Patient-Reported Outcomes Measurement Information System (PROMIS)-29 assesses seven health-related quality of life domains plus pain intensity. The objective was to examine PROMIS-29v2 validity and explore clinical associations in patients with SSc. English-speaking SSc patients in the Scleroderma Patient-centered Intervention Network Cohort from 26 sites in Canada, the USA and the UK completed the PROMIS-29v2 between July 2014 and November 2015. Enrolling physicians provided medical data. To examine convergent validity, hypotheses on the direction and magnitude of correlations with legacy measures were tested. For clinical associations, t -tests were conducted for dichotomous variables and PROMIS-29v2 domain scores. Effect sizes (ESs) were labelled as small (<0.25), small to moderate (0.25-0.45), moderate (0.46-0.55), moderate to large (0.56-0.75) and large (>0.75). There were 696 patients (87% female), mean ( s . d .) disease duration 11.6 (8.7) years, 57% with limited cutaneous subtype. Validity indices were consistent with seven of nine hypotheses (| r | =0.51-0.87, P < 0.001), with minor divergence for two hypotheses. Gastrointestinal involvement was associated with significantly worse outcomes for all eight PROMIS-29v2 domains (moderate or moderate to large ES in six of eight). Presence of joint contractures was associated with significant decrements in seven domains (small or small to moderate ESs). Skin thickening, diffuse cutaneous subtype and presence of overlap syndromes were significantly associated (small or small to moderate ESs) with five or six domains. This study further establishes the validity of the PROMIS-29v2 in SSc and underlines the importance of gastrointestinal symptoms and joint contractures in reduced health-related quality of life.

Using Optimal Test Assembly Methods for Shortening Patient-Reported Outcome Measures: Development and Validation of the Cochin Hand Function Scale-6: A Scleroderma Patient-Centered Intervention Network Cohort Study.

To develop and validate a short form of the Cochin Hand Function Scale (CHFS), which measures hand disability, for use in systemic sclerosis, using objective criteria and reproducible techniques. Responses on the 18-item CHFS were obtained from English-speaking patients enrolled in the Scleroderma Patient-Centered Intervention Network Cohort. CHFS unidimensionality was verified using confirmatory factor analysis, and an item response theory model was fit to CHFS items. Optimal test assembly (OTA) methods identified a maximally precise short form for each possible form length between 1 and 17 items. The final short form selected was the form with the least number of items that maintained statistically equivalent convergent validity, compared to the full-length CHFS, with the Health Assessment Questionnaire (HAQ) disability index (DI) and the physical function domain of the 29-item Patient-Reported Outcomes Measurement Information System (PROMIS-29). There were 601 patients included. A 6-item short form of the CHFS (CHFS-6) was selected. The CHFS-6 had a Cronbach's alpha of 0.93. Correlations of the CHFS-6 summed score with HAQ DI (r = 0.79) and PROMIS-29 physical function (r = -0.54) were statistically equivalent to the CHFS (r = 0.81 and r = -0.56). The correlation with the full CHFS was high (r = 0.98). The OTA procedure generated a valid short form of the CHFS with minimal loss of information compared to the full-length form. The OTA method used was based on objective, prespecified criteria, but should be further studied for viability as a general procedure for shortening patient-reported outcome measures in health research.

Validation of the Self-Efficacy for Managing Chronic Disease Scale: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Self-management programs for patients with chronic illnesses, including rheumatic diseases, seek to enhance self-efficacy for performing health management behaviors. No measure of self-efficacy has been validated for patients with systemic sclerosis (SSc; scleroderma). The objective of this study was to assess the validity and internal consistency reliability of the Self-Efficacy for Managing Chronic Disease (SEMCD) scale in SSc. English-speaking SSc patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort who completed the SEMCD scale at their baseline assessment between March 2014 and June 2015 were included. Patients were enrolled from 21 sites in Canada, the US, and the UK. Confirmatory factor analysis (CFA) was used to evaluate the factor structure of the SEMCD scale. Cronbach's alpha was calculated to assess internal consistency reliability. Hypotheses on the direction and magnitude of Pearson's correlations with psychological and physical outcome measures were formulated and tested to examine convergent validity. A total of 553 patients were included. CFA supported the single-factor structure of the SEMCD scale (Tucker Lewis Index = 0.99, comparative fit index = 0.99, root mean square error of approximation = 0.10). Internal consistency was high (α = 0.93), and correlations with measures of psychological and physical functioning were moderate to large (|r| = 0.48-0.67, P < 0.001), confirming study hypotheses. Scores from the SEMCD scale are valid for measuring self-efficacy in patients with SSc, and results support using the scale as an outcome measure to evaluate the effectiveness of self-management programs in SSc.