Shadi Gholizadeh

The Scleroderma Patient-centered Intervention Network (SPIN) Cohort: protocol for a cohort multiple randomised controlled trial (cmRCT) design to support trials of psychosocial and rehabilitation interventions in a rare disease context

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 interventions 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.

Psychosocial Aspects of Scleroderma.

Patients with systemic sclerosis (SSc; also called scleroderma) have to cope with not only the physical impacts of the disease but also the emotional and social consequences of living with the condition. Because there is no cure for SSc, improving quality of life is a primary focus of treatment and an important clinical challenge. This article summarizes significant problems faced by patients with SSc, including depression, anxiety, fatigue, sleep disruption, pain, pruritus, body image dissatisfaction, and sexual dysfunction, and describes options to help patients cope with the consequences of the disease.

Psychosocial aspects of systemic sclerosis.

Purpose of reviewIn 2010, an international consortium of researchers published a consensus agenda for research on psychosocial aspects of systemic sclerosis (SSc; scleroderma). The present review summarizes recent research on SSc-related depression and anxiety, fatigue and sleep, pain, pruritus, body image distress, sexual function, work disability, healthcare needs, psychosocial interventions, and psychoneuroimmunology. Recent findingsResearchers have used structured interviews to establish prevalence rates for clinical mood disorders in SSc, although anxiety remains understudied and distress may be a useful outcome to consider. Longitudinal research has identified predictors of fatigue. Research on body image distress suggests the importance of changes in the facial skin and hands. Studies have identified sexual function problems in women and men with SSc. A prospective study found that breathing problems and fatigue predicted workplace disability. A randomized controlled trial evaluating multidisciplinary care showed benefits for health-related quality of life. SummaryThere has been a recent expansion in studies of psychosocial aspects of SSc, and in the validation of psychosocial measures that can be used in clinics to identify problems and track outcomes; however, prospective studies remain scarce. To better address the psychosocial needs of persons with SSc, interventions need to be developed and tested via randomized controlled trials with power to detect clinically meaningful changes.

The many wounds of war: The association of service-related and clinical characteristics with problems with the law in Iraq and Afghanistan veterans

Previous research has demonstrated that veterans with posttraumatic stress disorder (PTSD) are at higher risk for aggression, hostility, and anger, potentially leading to problems with the law or disciplinary action while in the military. There have been calls for increased consideration of the unique aspects of the judicial system in legal matters with veterans involved. The bulk of the research so far has considered the link between PTSD, traumatic brain injury (TBI), and combat exposure, but little is known about the potential role of chronic pain and Military Sexual Trauma (MST) in a veterans experience of anger, aggression, and discipline while in the military and legal concerns. The present study used retrospective cross-sectional health screening data in a sample of 1250 Iraq and Afghanistan veterans to examine the associations of demographic, service-related, and clinical characteristics with self-reported legal problems. A total of 440 veterans (34.6%) endorsed having experienced some type of problems with the law. Independent logistic regression analyses demonstrated that those who screened positive for PTSD, MST, TBI, and clinical levels of pain were more likely to report legal problems; only the associations with positive PTSD (p=0.001) and MST (p=0.007) screens remained significant in multivariate regression analyses. The findings underscore the need for a thorough psychological evaluation when veterans are involved in the legal system with a special emphasis on factors prevalent to Iraq and Afghanistan veterans.

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.

Validation of the Body Concealment Scale for Scleroderma (BCSS): Replication in the Scleroderma Patient-centered Intervention Network (SPIN) cohort

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 Cronbachs 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.

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

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.

Etiology unknown: Qualitative analysis of patient attributions of causality in scleroderma

Background Questions about the etiology of disease can concern patients living with any chronic disease and may impact disease-related adjustment. These causal attributions may be of particular interest when individuals are living with diseases for which etiologies have not been definitively identified, such as scleroderma. This study qualitatively explored patient attributions of causality for scleroderma. Methods: Patients with confirmed diagnoses of scleroderma responded to an open-ended prompt. The cross-sectional sample of scleroderma patients (N = 114) was recruited through registries maintained at the University of California, Los Angeles and University of California, San Diego Schools of Medicine and the Virginia Mason Medical Center. Content analysis was used to analyze the qualitative data and group the responses via an inductively derived codebook using the text analysis tool Dedoose Version 4.5. Results: Patients provided a variety of possible causes for scleroderma, which grouped into seven themes: (1) stress, (2) environment, (3) genetics, (4) medical conditions or surgeries, (5) diet, (6) medications or substance use, and (7) spirituality. Conclusion: Patients’ causal attributions for scleroderma were varied, but many patients identified stress as a cause of scleroderma, often focusing on acute or chronic stressors that were present before disease onset. Identifying patient theories of causality for scleroderma can contribute to an increased understanding of disease-related behaviors and adjustment.

Factor structure and convergent validity of the Derriford Appearance Scale-24 using standard scoring versus treating 'not applicable' responses as missing data: A Scleroderma Patient-centered Intervention Network (SPIN) cohort study

Objective Valid measures of appearance concern are needed in systemic sclerosis (SSc), a rare, disfiguring autoimmune disease. The Derriford Appearance Scale-24 (DAS-24) assesses appearance-related distress related to visible differences. There is uncertainty regarding its factor structure, possibly due to its scoring method. Design Cross-sectional survey. Setting Participants with SSc were recruited from 27 centres in Canada, the USA and the UK. Participants who self-identified as having visible differences were recruited from community and clinical settings in the UK. Participants Two samples were analysed (n=950 participants with SSc; n=1265 participants with visible differences). Primary and secondary outcome measures The DAS-24 factor structure was evaluated using two scoring methods. Convergent validity was evaluated with measures of social interaction anxiety, depression, fear of negative evaluation, social discomfort and dissatisfaction with appearance. Results When items marked by respondents as ‘not applicable’ were scored as 0, per standard DAS-24 scoring, a one-factor model fit poorly; when treated as missing data, the one-factor model fit well. Convergent validity analyses revealed strong correlations that were similar across scoring methods. Conclusions Treating ‘not applicable’ responses as missing improved the measurement model, but did not substantively influence practical inferences that can be drawn from DAS-24 scores. Indications of item redundancy and poorly performing items suggest that the DAS-24 could be improved and potentially shortened.

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.