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Systemic sclerosis (SSc), a chronic systemic autoimmune disease, affects skin and internal organs compromising organ function and leading to significant morbidity and poor health-related quality of life (HrQoL). This cross-sectional study investigated whether HrQoL is influenced by trait emotional intelligence (TEI). Sixty patients with SSc (Female: 86.67%) completed the socio-demographic characteristics form, TEI Questionnaire Short-Form (TEIQue-SF), and Short-Form Health Survey (SF-36). Sixty healthy controls were also completed the TEIQue-SF. A series of multiple linear regression analyses with correlation matrix was used to analyze SF-36 domains as dependent variables with TEI domains (well-being, self-control, emotionality, sociability) as independent variables. The average age of participants was 57.3 ± 12.9 years with a mean disease duration of 7.7 ± 6.7 years. Patients differed from controls in the sociability domain of TEI. TEI global was found to affect the physical and mental component summaries (p < .001), and all 8 dimensions of the HrQoL (p < .001). Age, disease duration, and gastrointestinal manifestations were negatively associated with various components of SF-36. TEI was positively associated with all dimensions of HrQoL. Understanding the relationship between TEI and HrQoL dimensions is important for the support and empowerment of SSc patients, as well as the establishment and implementation of appropriate psychotherapeutic interventions., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet's syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.e., female sexual function index (FSFI), Beck depression inventory (BDI), body cathexis scale, and marital adjustment test (MAT) were performed. Scale scores were compared, and binary logistic regression was used to identify predictors for SD in the whole group. The total FSFI and body cathexis scores among the patient groups were significantly lower than those of the HCs (p < 0.001). Depression was significantly more frequent in the patient groups. MAT scores did not differ significantly between the study groups. Patients with SSc had the worst scores in each psychometric index, including MAT. Decreased body cathexis score [OR 0.974, 95% CI (0.957-0.991), p = 0.003] and low MAT score [OR 0.937, 95% CI (0.896-0.980), p = 0.005], and being diagnosed with SSc [OR 6.6, 95% CI (1.975-22.498), p = 0.002], SLE [OR 2.7, 95% CI (0.998-7.753), p = 0.050], and BS [OR 2.8, 95% CI (1.100-7.359), p = 0.031], were identified as independent predictors for SD. Body cathexis seems to be the most important independent predictor for SD, and the burden of SD appears heavier in patients with SSc, probably due to poor body image satisfaction., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Symptoms of anxiety increased early in the COVID-19 pandemic among people with systemic sclerosis (SSc) then returned to pre-pandemic levels, but this was an aggregate finding and did not evaluate whether vaccination may have contributed to reduced anxiety symptom levels. We investigated whether being vaccinated for COVID-19 was associated with reduced anxiety symptoms among people with SSc., Methods: The longitudinal Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 Cohort was launched in April 2020 and included participants from the ongoing SPIN Cohort and external enrollees. Participants completed measures bi-weekly through July 2020, then every 4 weeks afterwards through August 2022 (32 assessments). We used linear mixed models to evaluate longitudinal trends of PROMIS Anxiety 4a v1.0 anxiety domain scores and their association with vaccination., Results: Among 517 participants included in analyses, 489 (95%) were vaccinated by September 2021, and no participants were vaccinated subsequently. Except for briefly at the beginning, when few had received a vaccine, and end, when only 28 participants remained unvaccinated, anxiety symptom trajectories were largely overlapping. Participants who were never vaccinated had higher anxiety symptoms by August 2022, but there were no other differences, and receiving a vaccination did not appear to change anxiety symptom trajectories meaningfully., Conclusion: Vaccination did not appear to influence changes in anxiety symptoms among vulnerable people with SSc during the COVID-19 pandemic. This may be due to people restricting their behavior when they were unvaccinated and returning to more normal social engagement once vaccinated to maintain a steady level of anxiety symptoms., Competing Interests: Declaration of competing interest The authors have competing interests to declare., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: Facilitated self-management interventions have the potential to enhance resilience and well-being. We examined whether resilience is a mediator of improving physical and psychological symptoms for people with systemic sclerosis (SSc) who participated in a 12-week online peer-led symptom management intervention., Methods: We conducted a secondary data analysis from a randomized control trial comparing a peer health-coached intervention to a waitlist control. Participants completed the Connor-Davidson Resilience Scale, the Functional Assessment of Chronic Illness Therapy-Fatigue scale, and the Patient Reported Outcomes Measurement Information System measures of pain interference and depressive symptoms at the baseline and at weeks 6 and 12. Linear mixed effect regression models were used to assess the effect of intervention on changes in resilience. Causal mediation analyses were conducted to examine whether changes in resilience at week 12 mediated intervention effects on changes in fatigue, pain interference, and depressive symptoms at week 12., Results: One hundred and seventy-three eligible participants were enrolled. Participants in the intervention group reported improvements in resilience (P < 0.001). These changes in resilience mediated the intervention effects on fatigue with indirect effect of -1.41 (95% confidence interval [CI] -2.41 to -0.41), pain interference of -0.86 (95% CI -1.65 to -0.08), and depressive symptoms of -1.99 (95% CI -3.16 to -0.81)., Conclusion: For participants in the intervention who had positive improvements in their physical and psychological symptoms, increased resilience was a mechanism for these improvements. These findings support the importance of addressing resilience to improve symptoms in similar SSc interventions., (© 2024 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Objectives: The aim of this work is to review the existing literature regarding sexual and reproductive function of women affected by systemic sclerosis and to establish the impact of the disease on the gynaecological-obstetrical field., Methods: A systematic search has been conducted by means of PubMed, Cochrane, Google Scholar, until January 2024 by the keywords ''systemic sclerosis'', ''fertility'', "sexual dysfunction" and "pregnancy"., Results: Sexual dysfunction has been described in most of the studies. This could be related to dryness and dyspareunia, but also to the psychosocial impact of SSc on body and facial appearance, which impacts on social and sexual relationships. There is conflicting evidence regarding the influence of SSc and fertility. Before the 1980s pregnancies in these patients were rare. This could be linked to the satisfied reproductive desire before the onset of SSc, or to the fact that pregnancy was labelled as high-risk, leading to counsel against it in most patients. Recently, the evidence supporting infertility is conflicting. There is no certain theory on how the disease may interfere with reproductive function, but a possible linkage can be detected in a pro-inflammatory milieu which can impair the ovarian reserve., Conclusions: Women affected by SSc should be followed-up by a multidisciplinary team to prevent sexual dysfunction. Although there is no consensus on the impact of SSc on fertility, these patients should be provided with adequate pre-conceptional counselling and a strict follow-up in high-risk pregnancy units.

Objectives: People with systemic sclerosis (SSc) may find it challenging to obtain high-quality nutrition and diet information. Objectives were to evaluate (i) how commonly different information resources are used and (ii) perceived trustworthiness, accessibility, comprehensibility, and individualisation of resources., Methods: We administered the Scleroderma Patient-centred Intervention Network Nutrition Information Resources Survey to participants in an international cohort. Participants were asked if they had used 26 informational resources in four categories, including (i) health care providers, (ii) websites or social media, (iii) print materials, and (iv) events, and to rate each resource on trustworthiness, accessibility, comprehensibility, and individualisation (0 = not at all to 10 = completely)., Results: 727 participants completed the survey. Most (94%) had sought nutrition or diet information from at least one resource. The most-used category was health care providers (86%), followed by print materials (68%), websites or social media (66%), and events (43%). People who had used a resource generally rated it more favourably across all domains than those who had not. The highest-rated resources across domains were conventional health care providers (doctors, registered dieticians, nurses), SSc patient organisations, SSc support groups, and university or research institution websites., Conclusions: Respondents used many different diet and nutrition information resources. They preferred resources from conventional health care providers, affiliated with credible institutions (e.g., SSc patient organisations), or with personal connections (e.g., SSc support groups). Future research should address the limited evidence base on nutrition in SSc and assess the quality of information provided by different information resources.

Objective: The importance of early integration of palliative care in the management of complex multisystem diseases has been recognized. In this study, we aimed to quantify the need for specialist palliative care in patients with systemic sclerosis (SSc)., Methods: Using data from 875 patients enrolled in the Australian Scleroderma Cohort Study, we defined the need for palliative care as a high symptom burden at two or more consecutive study visits, at ≥50% of overall study visits, or at the study visit immediately before death. Symptoms of interest included breathlessness, fatigue, pain, depression, anxiety, constipation, and diarrhea. Logistic regression analyses evaluated the association between individual symptoms and SSc manifestations. Linear regression analysis evaluated the relationship between palliative care needs and quality of life (QoL) and function., Results: Almost three-quarters of patients (72.69%) met the threshold for specialist palliative care needs. Severe fatigue (54.17%) was most common, followed by breathlessness (23.66%) and severe constipation (21.14%). Concurrent severe symptoms were frequently observed. Severe breathlessness (coefficient [coef] -7.95, P < 0.01) and pain (coef -7.70, P < 0.01) were associated with the largest reductions in physical QoL. Severe mood symptoms were associated with the greatest reduction in mental QoL (coef -12.91, P < 0.01). Severe pain (coef 0.56, P < 0.01), breathlessness (coef 0.49, P < 0.01), and mood symptoms (coef 0.40, P < 0.01) had a significant impact on function., Conclusion: SSc is frequently associated with multiple severe symptoms that may be amenable to palliative care intervention. Given the strong association between symptom burden and impaired QoL targeted, effective symptom management in parallel with standard-of-care treatments may improve overall patient outcomes., (© 2024 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Background: Many individuals with systemic sclerosis (SSc) are at heightened risk for COVID-19 related morbidity and isolation due to interstitial lung disease, frailty, and immunosuppressant use. Minimal research has explored loneliness predictors in individuals with chronic illnesses during COVID-19. This study evaluated moderators of loneliness trajectories in individuals with SSc during COVID-19., Methods: Longitudinal data were analyzed across 30 timepoints from April 2020 to May 2022 from 775 adults in the Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 Cohort. Hierarchical linear modeling evaluated cross-level moderators of loneliness trajectories, including marital status, baseline number of household members, number of virtual or telephone one-on-one or virtual group conversations, number of hours spent enjoying in-person household conversations or activities, and satisfaction with quality of in-person household conversations (all in the past week). Level-1 moderation analyses assessed effects of conversation, activity, and satisfaction means and slopes over time., Results: Baseline values were not statistically significant moderators of loneliness trajectories. Higher mean (averaged over time) virtual or telephone one-on-one and in-person household conversations, in-person household activity, and in-person household conversation satisfaction were associated with lower loneliness trajectories (ps < .05). The relationship between in-person household conversation satisfaction and loneliness trajectory was statistically significantly but minimally attenuated over time (p < .001)., Conclusions: For people with SSc, higher mean conversation, activity, and satisfaction variables were associated with lower levels of loneliness during the pandemic, but changes in these social variables were generally not predictive of changes in loneliness., (© 2024. International Society of Behavioral Medicine.)

Objective: This study aimed to evaluate the quality of sleep in individuals with systemic sclerosis and its correlation with the quality of life and disability., Methods: This is a cross-sectional study, carried out in a tertiary service of a university hospital. Inclusion criteria were diagnosis of systemic sclerosis according to the criteria of the American College of Rheumatology/European League Against Rheumatism 2013 or the preliminary criteria of the American College of Rheumatology 1980, age ≥ 18 years; regularly monitored at the outpatient clinic of rheumatology. Clinical and demographic data of the patients were obtained through a structured interview and evaluation of the medical records. Sleep quality was assessed using the Pittsburgh Sleep Quality Index questionnaire, daytime sleepiness using the Epworth Sleepiness Scale, quality of life using 12-item short-form health survey, and disability using the scleroderma health assessment questionnaire., Results: A total of 50 patients with systemic sclerosis were included, with 92% female, mean age 48.9 years, mean disease duration 8.9 years, and 60% limited cutaneous form. Most systemic sclerosis patients (84%) have poor sleep quality and 20% have excessive daytime sleepiness. There was a significant negative correlation between Pittsburgh Sleep Quality Index and the physical and mental components of the 12-item short-form health survey (r=-0.42, p=0.003 and r=-0.43, p=0.002, respectively) and a positive correlation with the scleroderma health assessment questionnaire (r=0.52, p=<0.001)., Conclusion: This study showed that poor sleep quality is a very common finding among systemic sclerosis patients, and it negatively affects both the quality of life and the degree of disability. Sleep quality is an unmet need in patients with systemic sclerosis Poor sleep quality is very common in patients with systemic sclerosis Poor sleep quality correlated with worse quality of life and greater disability.

Objectives: To describe the severity and impact of gastrointestinal involvement in patients with systemic sclerosis (SSc) and identify associated factors., Patients and Methods: Non-controlled cross-sectional study of patients with SSc (2013 American College of Rheumatology/European League Against Rheumatism criteria). The main variables were severity of gastrointestinal involvement according to the University of California, Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 instrument (UCLA SCTC GIT 2.0) and dysphagia according to the Eating Assessment Tool-10 (EAT-10). We evaluated reflux, distension, diarrhoea, faecal soilage, constipation, emotional well-being and social functioning, as well as dysphagia. Clinical and epidemiological data were collected using the Mini Nutritional Assessment Short Form (MNA-SF) and the EuroQol-5D-3L. The degree of skin fibrosis was assessed using the modified Rodnan skin score (mRSS). Multivariate models were constructed to analyse factors associated with gastrointestinal involvement and dysphagia., Results: Of the 75 patients with SSc included, 58.7% had moderate, severe or very severe reflux, 57.4% had constipation according to UCLA SCTC GIT 2.0 and 49.7% had abdominal distension. Gastrointestinal symptoms interfered significantly with social functioning (42.7%) and emotional well-being (40.0%). Dysphagia (EAT-10≥3) was recorded in 52% of patients, and according to MNA-SF poor nutrition in 30.7%, and clear malnutrition requiring a nutritional intervention in 5.3%. Multivariate adjustment revealed an association between severity of gastrointestinal symptoms according to the mRSS (β=0.249; p=0.002) and Visual Analogue Scale 3-Level EuroQol-5D (VAS-EQ-5D-3L) (β=-0.302; p=0.001), whereas presence of dysphagia was associated with the mRSS (OR=2.794; p=0.015), VAS-EQ-5D-3L (OR=0.950; p=0.005) and malnutrition (MNA-SF≤7; OR=3.920; p=0.041)., Conclusions: Patients with SSc frequently present severe gastrointestinal symptoms. These are associated with poor quality of life, more severe skin involvement and malnutrition., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: A previous study using Scleroderma Patient-centered Intervention Network (SPIN) Cohort data identified five classes of people with systemic sclerosis (also known as scleroderma) based on patient-reported somatic (fatigue, pain, sleep) and mental health (anxiety, depression) symptoms and compared indicators of disease severity between classes. Across four classes ("low", "normal", "high", "very high"), there were progressively worse somatic and mental health outcomes and greater disease severity. The fifth ("high/low") class, however, was characterized by high disease severity, fatigue, pain, and sleep but low mental health symptoms. We evaluated resilience across classes and compared resilience between classes., Methods: Cross-sectional study. SPIN Cohort participants completed the 10-item Connor-Davidson-Resilience Scale (CD-RISC) and PROMIS v2.0 domains between August 2022 and January 2023. We used latent profile modeling to identify five classes as in the previous study and multiple linear regression to compare resilience levels across classes, controlling for sociodemographic and disease variables., Results: Mean CD-RISC score (N = 1054 participants) was 27.7 (standard deviation = 7.3). Resilience decreased progressively across "low" to "normal" to "high" to "very high" classes (mean 4.7 points per step). Based on multiple regression, the "high/low" class exhibited higher resilience scores than the "high" class (6.0 points, 95% confidence interval [CI] 4.9 to 7.1 points; standardized mean difference = 0.83, 95% CI 0.67 to 0.98)., Conclusions: People with worse disease severity and patient-reported outcomes reported substantially lower resilience, except a class of people with high disease severity, fatigue, pain, and sleep disturbance but positive mental health and high resilience., Competing Interests: Declaration of competing interest The authors have no competing interests to report., (Copyright © 2023. Published by Elsevier Inc.)

Objective: 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., Methods: 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)., Results: 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., Conclusion: The CD-RISC-10 is a valid and reliable measure of resilience in SSc, with score comparability across English and French versions., (© 2023 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Background: Systemic sclerosis (SSc) is a chronic autoimmune disease that affects the connective tissues and leads to physical, emotional, and social challenges for patients. Evaluating health-related quality of life (HRQoL) with a disease-specific tool may be preferable for improving patient care and treatment outcomes. The aim of this study was to translate the Systemic Sclerosis Quality of Life Questionnaire (SScQoL) into Turkish and to investigate its psychometric properties., Methods: Eighty-six patients with SSc (mean age 51.8 ± 11.7 years, 80 females) participated in the study. Convergent validity was explored by correlation analyses between Turkish SScQoL and Short-Form 36 (SF-36), European Quality of Life Survey-5 Dimensions (EQ-5D), EQ-5D Visual Analog Scale (EQ-VAS), and Scleroderma Health Assessment Questionnaire (SHAQ). Cronbach's alpha was calculated to test internal consistency. Turkish SScQoL was readministered after 7-14 days to fifty-eight patients for determining test re-test reliability. Intraclass correlation coefficients in 95% confidence interval (ICCs [95%CI]) were calculated to examine the agreement between two assessments. Values greater than 15% and an absolute skewness value < 1 were recognized as the presence of a floor or ceiling effect., Results: SScQoL correlated significantly with SF-36 subdomains (r = -0.347 to -0.618, p < 0.01), EQ-5D (r = -0.535, p < 0.01), EQ-VAS (r = -0.636, p < 0.01), and SHAQ global score (r = 0.521, p < 0.01). SScQoL demonstrated excellent internal consistency (Cronbach's alpha = 0.917), and good to excellent test-retest reliability (ICC [95%CI] = 0.85 [0.76-0.91]). No floor/ceiling effects were observed., Conclusion: The Turkish version of SScQoL seems to have adequate psychometric properties and can be used to evaluate HRQoL in clinical and research settings. Key points • Turkish version of SScQoL is a valid and reliable tool for measuring health-related quality of life of patients with systemic sclerosis. • SScQoL is the only diseases-specific quality of life measurement for systemic sclerosis available in Turkish. • Patients with limited and diffuse SSc seem to be similar in terms of self-reported health-related quality of life., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objectives: People with systemic sclerosis (SSc) are vulnerable in COVID-19 and face challenges related to shifting COVID-19 risk and protective restrictions. We evaluated mental health symptom trajectories in people with SSc through March 2022., Methods: The longitudinal Scleroderma Patient-centred Intervention Network (SPIN) COVID-19 cohort was launched in April 2020 and included participants from the ongoing SPIN Cohort and external enrolees. Analyses included estimated means with 95% CIs for anxiety and depression symptoms pre-COVID-19 for ongoing SPIN Cohort participants and anxiety, depression, loneliness, and fear of COVID-19 for all participants across 28 COVID-19 assessments up to March 2022. We conducted sensitivity analyse including estimating trajectories using only responses from participants who completed >90% of items for ≥21 of 28 possible assessments ("completers") and stratified analyses for all outcomes by sex, age, country, and SSc subtype., Results: Anxiety symptoms increased in early 2020 but returned to pre-COVID-19 levels by mid-2020 and remained stable through March 2022. Depression symptoms did not initially change but were slightly lower by mid-2020 compared to pre-COVID-19 and were stable through March 2022. COVID-19 fear started high and decreased. Loneliness did not change across the pandemic. Results were similar for completers and for all subgroups., Conclusions: People with SSc continue to face COVID-19 challenges related to ongoing risk, the opening of societies, and removal of protective restrictions. People with SSc, in aggregate, appear to be weathering the pandemic well, but health care providers should be mindful that some individuals may benefit from mental health support.

Objective: Perceived cognitive dysfunction is prevalent in patients with systemic sclerosis (SSc) but not well understood. This study aimed to examine potential factors associated with perceived cognitive function and to investigate the contributions of perceived cognitive function and symptoms to functional measures., Methods: A cross-sectional survey was conducted among patients with SSc (n = 106). Participants were mainly female (84%) and White (82%). Perceived cognitive function, symptoms, and functional measures were assessed with Patient-Reported Outcomes Measurement Information System (PROMIS) measures. A multivariable regression was conducted to identify factors associated with perceived cognition. Hierarchical linear regressions examined the unique contributions of perceived cognitive function and symptoms to social participation and physical function., Results: Fifty-nine (56%) patients with SSc perceived mild-to-severe cognitive dysfunction. Being on work disability and having more fatigue were both significantly associated with perceived cognitive dysfunction. When examining the contributions of cognition and other symptoms to functional measures, self-reported cognition became nonsignificant after fatigue and pain were entered into the regression model., Conclusion: Being on work disability and having more fatigue were most highly associated with perceived cognitive dysfunction in patients with SSc. Unlike fatigue and pain, perceived cognitive function was not independently associated with functional measures. Nonetheless, future research should disentangle cognitive function and other symptoms, as well as their effects on daily activities, in SSc., (© 2022 American College of Rheumatology.)

Objectives: This study used a qualitative approach to explore how people with SSc experience cognitive changes and how cognitive difficulties impact their functioning., Methods: Four 90-min focus groups of adults with SSc and self-reported changes in cognition were recruited from a SSc research registry and targeted social media. A focus group guide elicited information from participants via open-ended questions. Content analysis was conducted using grounded theory methodology., Results: There were 20 participants (mean age = 55.5 (11.4) years) comprising 16 (80%) females, 14 (70%) Caucasians, and 11 (55%) people with diffuse cutaneous SSc. Study themes included cognitive difficulties as part of daily life experience, impact of cognitive difficulties on daily life functioning, coping strategies and information seeking. Participants used different terms to describe their experience of cognitive difficulties, and most encountered deficits in short-term memory, language difficulties, decreased executive function, difficulties with concentration and focus, and slow processing speed. Participants expressed frustration with their cognitive difficulties and used coping strategies to lessen their impact. Participants were uncertain about the causes and wanted to understand factors contributing to cognitive difficulties as well as how to manage them., Conclusion: Participants with SSc reported cognitive difficulties that had a substantial negative impact on their lives. Improved understanding of cognitive changes could subsequently facilitate development of relevant therapeutic interventions or educational programmes for symptom self-management to reduce impact of cognitive difficulties in people with SSc., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Patients' objectives and experiences must be core to the study and management of chronic diseases, such as SSc. Although patient-reported outcomes are attracting increasing attention, evaluation of the impact of disease on the overall subjective well-being, equivalent to 'happiness', is remarkably lacking., Objectives: To examine the determinants of happiness and quality of life in patients with SSc, with emphasis on disease features and personality traits., Methods: Observational, cross-sectional multicentre study, including 142 patients, with complete data regarding disease activity, disease impact, personality, health-related quality of life (HR-QoL) and happiness. Structural equation modelling was used to evaluate the association between the variables., Results: The results indicated an acceptable fit of the model to the data. Perceived disease impact had a significant negative direct relation with HR-QoL (β = -0.79, P < 0.001) and with happiness (β = -0.52, P < 0.001). Positive personality traits had a positive relation with happiness (β = 0.36, P = 0.002) and an important indirect association upon QoL (β = 0.43) and happiness (β = 0.23). Perceived disease impact is influenced by body image, fatigue and SSc-related disability to a higher degree (β = 0.6-0.7) than by disease activity (β = 0.28) or form (β = 0.17). Impact of disease had a much stronger relation with HR-QoL than with happiness., Conclusions: The results suggest that treatment strategies targeting not only disease control but also the mitigation of relevant domains of disease impact (body image, fatigue, global disability) may be important to improve patients' experience of the disease. The reinforcement of resilience factors, such as positive psychological traits, may also play a contributory role towards better patient outcomes., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To evaluate choroidal thickness (CT), corneal parameters, and scleral thickness (ST) in patients with systemic sclerosis (SSc) and to determine their relationship with disease-related quality of life (QoL)., Methods: The study included 38 patients with SSc and 40 healthy controls. A detailed ocular examination was performed on all participants. Corneal parameters such as K1, K2, Km, corneal volume (CV), central corneal thickness (CCT), and ST at a distance of 1000, 2000, and 3000 μm from the scleral spur were measured. CT was measured at five points, including the subfoveal area and the temporal and nasal points at radii of 750.0 and 1500.0 μm. The scleroderma health assessment questionnaire (SHAQ) was administered to SSc patients to investigate the disease-related QoL., Results: Individuals with SSc had thicker ST at all distances from the scleral spur (P=0.008, P=0.001, P=0.002, respectively). All corneal parameters were significantly lower in the SSc group than in the control group (P < 0.05). Moreover, SSc patients had significantly lower median CT at N750.0, N1500.0, T750.0, and T1500.0 points and thinner subfoveal CT than healthy controls (P < 0.05). There was a weak-moderate negative correlation between ST and the components of the SHAQ scale and SHAQ-global., Conclusion: Despite not having ocular involvement, SSc patients had thicker ST but thinner CT and corneal parameters than healthy controls. This may indicate subclinical inflammation in patients with SSc. Only ST was affected by organ involvement and QoL among the ocular parameters., (© 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

The outbreak of SARS-CoV-2 has changed the habits and lives of people worldwide. Patients affected by systemic sclerosis (SSc) experienced constant fear because of their immunocompromised status. The aim of this study was to investigate the prevalence of SARS-CoV-2 infection and to analyze the lifestyle changes in a single-center cohort of SSc patients and if these changes were more severe than in the general population. During the Italian lockdown, we supplied two surveys to our 184 SSc patients. In the first one, filled by 110 patients, we asked if SARS-CoV-2 had infected them or if they experienced signs and symptoms consistent with COVID-19. The second survey, performed by 79 SSc patients and 63 healthy subjects, included questions about the lifestyle adopted during this specific period. Among our patients, COVID-19 was diagnosed only in one case, while three other subjects reported signs and symptoms suggestive for the disease. Regarding the second survey, our patients greatly changed their lifestyle during the pandemic, adopting more restrictive isolation measures, because of their awareness of frailty. To date, we do not dispose of enough data to speculate about the risk of COVID-19 among immunocompromised patients, although in our SSc patients their frailty seems to have been their shelter. Pending more accurate epidemiological studies, it is essential to share as much data as possible to better understand the impact of COVID-19 on SSc patients' health. Key points • The lifestyle adopted by SSc patients during the first months of COVID-19 pandemic was characterized by more stringent isolation rules than general population. • The prudential behavior of patients with SSc during Italian lockdown should be considered as a possible bias when analyzing the risk of SARS-CoV-2 disease in these subjects, as well as a protective factor against infection.

Objective: Fatigue is consistently ranked as one of the most problematic symptoms of systemic sclerosis (SSc), but the impact of fatigue on daily life is not well characterized. The purpose of this study was to examine the contribution of fatigue to deficits in social participation, functioning, and quality of life., Methods: Baseline data from a sample undertaking a clinical trial were utilized (n = 267). Fatigue, pain interference, depressive symptoms, physical function, and social participation were assessed by measures from the Patient-Reported Outcomes Measurement Information System. Hierarchical linear regressions were performed to determine the unique contribution of fatigue to social participation, physical function, and quality of life above and beyond the effects of demographic and clinical variables, pain interference, and depressive symptoms., Results: The sample was predominantly female (91%), with an average age of 53.7 years, average disease duration of 9 years, and a mean fatigue T score of 58.7. Of all outcomes, fatigue was most strongly associated with deficits in social participation, explaining 48% of the variance beyond demographic and clinical factors, which is similar to the amount of variance contributed by pain interference and depressive symptoms combined (49%). Fatigue also accounted for significant amounts of variance in physical function and quality of life (R 2 = 0.27 and 0.33, respectively) above and beyond the effects of demographic and clinical factors., Conclusion: Fatigue is an important clinical problem in SSc and is strongly associated with decreased participation in social roles and activities. Rehabilitation interventions that focus on fatigue management may be necessary to maximize participation., (© 2019, American College of Rheumatology.)

Objective: Few studies have examined how workplace support can address work productivity loss among individuals with systemic sclerosis (SSc). The objective was to 1) examine the relationship between unmet workplace support needs and work productivity loss among workers with SSc, and 2) determine whether SSc symptom severity, fatigue, active disease periods, and workplace activity limitations mediate the relationship between unmet workplace support needs and work productivity loss., Methods: A cross-sectional survey was conducted of employed individuals with SSc who were recruited through rheumatology clinics. Information on work productivity loss (i.e., absenteeism, presenteeism, job disruptions) and the need, availability, and use of workplace supports was collected. SSc symptom severity (e.g., workplace activity limitations, active disease periods, fatigue, and overall SSc symptom severity) and demographic, health, and work context characteristics were collected. Three Bayesian path models examined the association between unmet workplace support needs and each work productivity loss outcome. SSc symptom severity variables were examined as mediators in each model., Results: A total of 110 employed participants were recruited (mean ± SD ages 49 ± 12.9 years). More than three-fourths of participants were female (77%) and worked full-time (77%). The most needed workplace supports included extended health benefits (84%), special equipment (63%), and flextime (59%). Additionally, 61% reported unmet workplace support needs. Path models indicated that indirect relationships between unmet workplace support needs and work productivity loss were significant. For all models, workplace activity limitations mediated the relationship between unmet workplace support needs and productivity loss., Conclusion: To foster productive employment of individuals with SSc, interventions need to address symptom severity and meet workplace support needs., (© 2019, American College of Rheumatology.)

Background/objectives: We hypothesized that emotional distress in systemic sclerosis (SSc) patients with moderate to severe gastrointestinal (GI) dysfunction is associated with dysautonomia. We sought to determine (1) the clinical characteristics associated with emotional distress in SSc, (2) the odds of having dysautonomia in those with emotional distress, and (3) whether GI dysautonomia, as measured by the Survey of Autonomic Symptoms (SAS), correlates with GI dysautonomia on the Composite Autonomic Symptom Score-31 (COMPASS-31)., Methods: Clinical and demographic features from our prospective cohort study were compared among SSc patients with and without GI-associated emotional distress (University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 well-being subscale >0.5 or ≤0.5) in cross-sectional analysis. Covariates/confounders independently associated with emotional distress were used to construct multivariable logistic regression models. The COMPASS-31 and SAS GI subdomains were compared with Spearman correlation., Results: Forty-six patients with SSc were enrolled in the study. In univariate analyses, age (odds ratio [OR], 1.06; p = 0.026), severity of GI dysautonomia (COMPASS-31: OR, 1.41; p = 0.003), anti-centromere (A/B) antibodies (OR, 3.60; p = 0.044), and anti-PM-Scl (75/100) antibodies (OR, 0.15; p = 0.035) were associated with emotional distress. In the adjusted model, those with more severe GI dysautonomia remained more likely to have emotional distress (OR, 1.85; p = 0.026); those with anti-PM-Scl (75/100) antibodies were less likely to have emotional distress (OR, 0.03; p = 0.031). The SAS and COMPASS-31 GI subdomains moderately correlated (ρ = 0.68, p < 0.001)., Conclusions: In SSc, increased symptom burden related to GI dysautonomia is associated with emotional distress. Multidisciplinary approaches addressing both the physical and emotional needs of the SSc patient may be warranted to optimize patient care., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

Background: 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., Objectives: 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., Methods: 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., Results: 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., Discussion: 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., Competing Interests: The authors do not have any conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: No studies have reported mental health symptom comparisons prior to and during COVID-19 in vulnerable medical populations., Objective: To compare anxiety and depression symptoms among people with a pre-existing medical condition and factors associated with changes., Methods: Pre-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., Results: Mean 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., Conclusions: Anxiety symptoms, but not depression symptoms, increased dramatically during COVID-19 among people with a pre-existing medical condition., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Fear associated with medical vulnerability should be considered when assessing mental health among individuals with chronic medical conditions during the COVID-19 pandemic. The objective was to develop and validate the COVID-19 Fears Questionnaire for Chronic Medical Conditions., Methods: Fifteen initial items were generated based on suggestions from 121 people with the chronic autoimmune disease systemic sclerosis (SSc; scleroderma). Patients in a COVID-19 SSc cohort completed items between April 9 and 27, 2020. Exploratory factor analysis (EFA) and item analysis were used to select items for inclusion. Cronbach's alpha and Pearson correlations were used to evaluate internal consistency reliability and convergent validity. Factor structure was confirmed with confirmatory factor analysis (CFA) in follow-up data collection two weeks later., Results: 787 participants completed baseline measures; 563 of them completed the follow-up assessment. Ten of 15 initial items were included in the final questionnaire. EFA suggested that a single dimension explained the data reasonably well. There were no indications of floor or ceiling effects. Cronbach's alpha was 0.91. Correlations between the COVID-19 Fears Questionnaire and measures of anxiety (r = 0.53), depressive symptoms (r = 0.44), and perceived stress (r = 0.50) supported construct validity. CFA supported the single-factor structure (χ 2 (35) = 311.2, p < 0.001, Tucker-Lewis Index = 0.97, Comparative Fit Index = 0.96, Root Mean Square Error of Approximation = 0.12)., Conclusion: The COVID-19 Fears Questionnaire for Chronic Medical Conditions can be used to assess fear among people at risk due to pre-existing medical conditions during the COVID-19 pandemic., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Depressive symptoms are common in rheumatic diseases and influence patients' quality of life. The Patient Health Questionnaire-9 (PHQ-9), which assesses symptoms of depression, is valid in English in patients with systemic sclerosis (SSc). However, the measurement properties of the PHQ-8 (short version of the PHQ-9) have not been evaluated in Swedish patients with SSc., Objective: To investigate different aspects of validity and reliability of the PHQ-8 in Swedish (PHQ-8 Swe) for individuals with SSc., Methods: A total of 101 patients with SSc participated. Content validity was evaluated via interviews of 11 patients and 10 health professionals. Construct validity, internal consistency test-retest reliability, and floor/ceiling effects were evaluated in 90 patients., Results: Content validity was satisfactory, but some linguistic adjustments were made. Confirmatory factor analysis supported a better fit for a two-factor structure. Moderate-to-strong correlations were found between the PHQ-8 Swe and scleroderma HAQ including VAS (r s  = 0.4-0.7); Multidimensional Assessment of Fatigue (r s  = 0.7); RAND-36 subscales (r s  = - 0.5 to - 0.8); and lung disease severity (Medsger scores) (r s  = 0.4). There were weak correlations (r s  = <0.4) between the PHQ-8 Swe and modified Rodnan skin score; and vascular, heart, and kidney disease severity. Cronbach's alpha was 0.85, corrected item-to-total correlations were >0.40, and the ICC for the total score was 0.83. No floor/ceiling effects were found., Conclusion: The PHQ-8 Swe has satisfactory content validity and sufficient reliability in patients with in majority limited SSc. It is more strongly associated with self-reported disability, pain, disease interferences with daily activities, fatigue, and quality of life than with disease severity, except for a moderate association with lung severity.

Objective: Digital ulcers (DUs) are a major cause of disease-related morbidity and are a difficult-to-treat vascular complication of systemic sclerosis (SSc). Demonstrating treatment efficacy has traditionally focused on clinician assessment of DUs alone. No existing patient-reported outcome (PRO) instrument captures the multifaceted impact of SSc-DU. We report the findings of a multicenter qualitative research study exploring the patient experience of SSc-DU., Methods: Patient focus groups were conducted across 3 scleroderma units, following a topic guide devised by SSc patients, experts, and experienced qualitative researchers. A purposive sampling framework ensured that the experiences of a diverse group of patients were captured. Focus groups were audio recorded, and information was transcribed, anonymized, and analyzed using inductive thematic analysis. We continued focus groups until thematic saturation was achieved., Results: Twenty-nine SSc patients with a history of DU disease participated in 4 focus groups across the UK (Bath, Manchester, and London). Five major interrelated themes (and subthemes) were identified that encompass the patient experience of SSc-DU: disabling pain and hypersensitivity; deep and broad-ranging emotional impact; impairment of physical and social activity; factors aggravating occurrence, duration, and impact; and mitigating, managing, and adapting., Conclusion: The patient experience of SSc-DU is multifaceted and comprises a complex interplay of experiences associated with significant pain and morbidity. Patient experiences of SSc-DU are not captured using existing SSc-DU outcomes. Our findings will inform the development of a novel PRO instrument to assess the severity and impact of SSc-DU for use in future SSc-DU clinical trials., (© 2019, American College of Rheumatology.)

Objectives: In severe rapidly progressive SSc, autologous haematopoietic stem cell transplantation (AHSCT) allows significant improvements in overall and event-free survival. We undertook this study to identify, appraise and synthesize the evidence on health-related quality of life (HRQoL) before and after AHSCT for SSc., Methods: We performed a systematic review of the literature, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, in PubMed and ScienceDirect from database inception to 1 February 2019. All articles with original HRQoL data were selected., Results: The search identified 1080 articles, of which 8 were selected: 3 unblinded randomized controlled trials [American Scleroderma Stem Cell versus Immune Suppression Trial (ASSIST), Autologous Stem Cell Transplantation International Scleroderma, Scleroderma: Cyclophosphamide or Transplantation), 3 uncontrolled phase I or II trials and 2 cohort studies. HRQoL data from 289 SSc patients treated with AHSCT and 125 treated with intravenous CYC as a comparator with median 1.25-4.5 years follow-up were included. HRQoL was evaluated with the HAQ Disability Index (HAQ-DI; 275 patients), the 36-item Short Form Health Survey (SF-36; 249 patients) and the European Quality of Life 5-Dimensions questionnaire (EQ-5D; 138 patients). The quality of the studies was moderate to low. AHSCT was associated with significant improvement in the HAQ-DI (P = 0.02-<0.001), SF-36 Physical Component Summary score (P = 0.02-<0.0001) and EQ-5D index-based utility score (P < 0.001). The SF-36 Mental Component Summary score improved in the ASSIST (n = 19) and one small retrospective cohort (n = 30 patients, P = 0.005) but did not improve significantly in 2 randomized controlled trials (n = 200 patients, P = 0.1-0.91)., Conclusion: AHSCT in severe SSc patients is associated with significant and durable improvement in physical HRQoL., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Purpose: Our study aimed to identify caregiver characteristics (gender, age, occupational status, educational attainment, relation to care recipient), care recipient characteristics (age, disease subtype), and caregiving factors (hours of care, perceived caregiving burden) associated with symptoms of depression among informal caregivers of persons with systemic sclerosis (also known scleroderma).as Materials and methods: A questionnaire was developed and administered online from December 2016 to June 2017 to informal caregivers of people with scleroderma, including the Patient Health Questionnaire-9 to assess depressive symptoms. Multiple linear regression was used to identify factors associated with depressive symptoms. Results: Caregivers ( n  = 202) were 60.9% male. Average age was 57.2 years (standard deviation = 14.4 years). Most caregivers were partners (72.3%), children (11.9%), or parents (7.4%), of care recipients. Self-reported caregiving burden (standardized regression coefficient [Formula: see text] = 0.54, p  < 0.001) and hours of care per week ([Formula: see text] = 0.17, p  = 0.005) were significantly associated with greater symptoms of depression. Conclusions: Depressive symptoms were primarily associated with caregiving burden among a sample of scleroderma caregivers. There is need to develop interventions targeting caregivers in scleroderma. Rehabilitation professionals should consider the specific needs of scleroderma caregivers and should provide or refer to support services as appropriate.Implications for RehabilitationMost people diagnosed with scleroderma are cared for by an informal caregiver and the majority of these caregivers experience mild symptoms of depression.For caregivers that experience moderate to severe symptoms of depression, the ability to provide care to a loved one with scleroderma may be more difficult.Rehabilitation professionals should be aware of the burden faced by caregivers of persons with scleroderma and provide support services or referrals as appropriate.

Introduction: The aim of this study was to evaluate the associations between malnutrition and the clinical features of the disease and depression in patients with systemic sclerosis (SSc)., Method: Patients with SSc who were followed up in our clinic were enrolled in the study. Malnutrition risk was assessed using the Malnutrition Universal Screening Tool (MUST). Skin involvement was assessed using the modified Rodnan skin score (mRSS) and interincisal distance (ID) measurements were used to assess the maximal mouth opening capacity. The Beck Depression Inventory (BDI) was used for measuring the severity of depression., Results: Ninety-eight patients with SSc (84.7% women; mean age 52.67 ± 11.26 years) were included in the study. According to the MUST scores, 61.2%, 15.3%, and 23.5% of patients had low, medium, and high risk for malnutrition, respectively. The mRSS was significantly higher in the group with high malnutrition risk compared with low-risk group (p = 0.014). Malnutrition risk was associated with interstitial lung disease and bowel involvement (p = 0.044 and p = 0.021, respectively). Interincisal distance was lower in the group with high malnutrition risk compared with the low-risk group (p = 0.003). Malnutrition risk was higher in patients who had mild-to-severe depressive symptoms than in those without (p = 0.012). Interincisal distance and bowel involvement were the most relevant factors for malnutrition., Conclusions: The risk of malnutrition is increased in patients with SSc. In our study, microstomia and bowel involvement were the most relevantly associated factors with malnutrition., Key Points: • The risk of malnutrition is increased in patients with systemic sclerosis (SSc). • Microstomia and bowel involvement are found to be the most important factors associated with malnutrition. • Depressive symptoms are seen frequently among patients with SSc, and depression seems to be one of the etiologic factors or the result of malnourishment in SSc. • Assessment of nutritional status and the presence of depression should be a part of routine clinical visits of patients with SSc.

Background: Some people with rare diseases rely on peer-led support groups for disease-specific education and emotional and practical support. Systemic sclerosis (SSc), or scleroderma, is a rare autoimmune connective tissue disease. Many people with SSc cannot access support groups, and, when support groups exist, they may not be sustained due to challenges that could be addressed via leader training. The Scleroderma Patient-centered Intervention Network (SPIN), along with SSc patient organization partners, developed a training program for SSc patient support group leaders, the Scleroderma Support group Leader EDucation (SPIN-SSLED) Program. We recently completed a feasibility trial in which we successfully delivered the program to two groups of support group leaders who reported a high level of satisfaction with the program and its delivery. The primary objective of the full-scale SPIN-SSLED trial is to evaluate the effect of the program on support group leaders' self-efficacy for carrying out their leadership role. Secondary objectives include evaluating effects on leader burnout, leader satisfaction (participation efficacy), and emotional distress., Methods/design: The SPIN-SSLED trial is a pragmatic randomized controlled trial (RCT) in which 180 support group leaders will be randomly allocated to training groups of 6 participants each or to a waitlist control. We will use a partially nested RCT design to reflect dependence between individuals in training groups, but not in the waitlist control. Participants allocated to the training program will receive the 13-module SPIN-SSLED Program, delivered via webinar over the course of 3 months in weekly 60-90-min sessions. The primary outcome is leader self-efficacy, measured by the Scleroderma Support Group Leader Self-efficacy Scale post-intervention. Secondary outcomes are leader self-efficacy at 3 months post-intervention, and leader burnout, volunteer job satisfaction (participation efficacy), and emotional distress post-intervention and at 3 months post-intervention., Discussion: The SPIN-SSLED trial will test whether a training program for SSc patient support group leaders increases the self-efficacy of group leaders to carry out leadership tasks. The program has the potential to significantly improve the effectiveness and sustainability of existing SSc support groups, to increase the number of available support groups, and to be adapted for other chronic diseases., Trial Registration: ClinicalTrials.gov, NCT03965780. Registered on 29 May 2019.

Introduction: Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs, leading to their failure and disturbances in the morphology and function of blood vessels. The disease affects people in different ways, and identifying how the difficulties and limitations are related to quality of life may contribute to designing helpful interventions. The aim of this study was to identify factors associated with quality of life in people with SSc., Methods: This was a cross-sectional study conducted in 11 rheumatic centres in Poland. Patients diagnosed with SSc were included. Quality of life was measured using the SSc Quality of Life Questionnaire (SScQoL). The following candidate factors were entered in preliminary multivariable analysis: age, place of residence, marital status, occupational status, disease type, disease duration, pain, fatigue, intestinal problems, breathing problems, Raynaud's symptoms, finger ulcerations, disease severity, functional disability, anxiety and depression. Factors that achieved statistical significance at the 10% level were then entered into a final multivariable model. Factors achieving statistical significance at the 5% level in the final model were considered to be associated with quality of life in SSc., Results: In total, 231 participants were included. Mean age (SD) was 55.82 (12.55) years, disease duration 8.39 (8.18) years and 198 (85.7%) were women. Factors associated with quality of life in SSc were functional disability (β = 2.854, p < 0.001) and anxiety (β = 0.404, p < 0.001). This model with two factors (functional disability and anxiety) explained 56.7% of the variance in patients with diffuse SSc and 73.2% in those with localized SSc., Conclusions: Functional disability and anxiety are significantly associated with quality of life in SSc. Interventions aimed at improving either of these factors may contribute towards improving the quality of life of people with SSc.

Purpose: The purpose of this study was to explore challenges faced by patients with systemic sclerosis, also called scleroderma, in coping with their disease and the strategies they used to face those challenges. Method: Five focus groups were held with scleroderma patients (4 groups, n  = 34) and health care professionals who have experience treating scleroderma (1 group, n  = 8). Participants' discussions were recorded, transcribed and analyzed using thematic analysis. Results: Participants reported challenges accessing information (e.g., knowledgeable specialists), dealing with negative emotions (e.g., stress due to misunderstandings with loved ones), and accessing resources (e.g., helpful products or devices). Strategies for overcoming challenges were also discussed (e.g., advocating for own needs). Conclusion: When faced with significant challenges while coping with scleroderma, patients develop strategies to manage better and improve their quality of life. To help them cope, patients would benefit from easier access to supportive interventions, including tailored scleroderma self-management programs. Although the challenges experienced by patients with scleroderma are unique, findings from this study might help better understand patients' perspectives regarding coping and disease management for other chronic diseases as well. Implications for Rehabilitation People living with rare diseases, including the rare autoimmune disease scleroderma, face unique challenges and often do not have access to disease-specific educational or other support resources. People with scleroderma report that they face challenges in accessing information, including knowledgeable healthcare providers; managing difficult social interactions and negative emotions; and accessing resources. Strategies employed by scleroderma patients to overcome these challenges include seeking connections to other people with scleroderma or scleroderma patient organizations, actively seeking out local resources, and learning to communicate and advocate more effectively. Rehabilitation professionals can support people with scleroderma by providing them with information on connecting with scleroderma patient organizations or by facilitating local patient support networks.

Systemic sclerosis (SSc) is a chronic, systemic disease characterized by fibrosis of the skin and internal organs, vasculopathy, and auto-immune activation. On the top of severe organ involvement such as interstitial lung and myocardial fibrosis, pulmonary hypertension, and renal crisis, individuals diagnosed with SSc may suffer from a number of comorbidities. This is a narrative review according to published recommendations and we searched the online databases MEDLINE and EMBASE using as key words the following terms: systemic sclerosis, scleroderma, myocardial fibrosis in combination with micro- and macro-vascular disease, cardiac involvement, atherosclerosis, cardiovascular disease and coronary arteries, infections, cancer, depression, osteoporosis, and dyslipidemia. Although data are usually inconclusive it appears that comorbidities with significant impact on life expectancy, namely cardiovascular disease, infections, and cancer as well as phycological disorders affecting emotional and mental health are highly prevalent in SSc population. Thereafter, the aim of this review is to summarize the occurrence and the clinical significance of such comorbidities in SSc population and to discuss how rheumatologists can incorporate the management of these conditions in daily clinical practice.

Background: In routine clinical practice, healthcare professionals draw little attention to pain in patients with systemic sclerosis (SSc). Pain has adverse effects on functional ability, social and emotional wellbeing., Aims: This study aims to assess the frequency and severity of different types of pain in patients with SSc, and the relationship of pain with disease status, depression and quality of life., Design: Consecutive patients with SSc were included in this cross-sectional study. Patients with previously diagnosed painful diseases or conditions (other rheumatic diseases, angina pectoris, neurological disorders, etc.) were excluded., Settings: Patients, who visited our rheumatology outpatient clinic from February to November 2016, participated in this study., Participants/subjects: 42 consecutive patients with SSc (38 women and 4 men), mean age 56.5 years, mean disease duration 9.5 years, were included., Methods: All patients filled in a questionnaire, to indicate the presence or absence of some predefined pain syndromes. Disease status was assessed using the Scleroderma Assessment Questionnaire (SAQ), symptoms of depression by the Beck's Depression Inventory (BDI), whilst the quality of life was evaluated using the EuroQol questionnaire., Results: It was found that 92.9% of SSc patients suffer from different types of pain, and 45.2% of patients have pain every day. Joint pain was the most common type of pain, present in 78.6% of patients, followed by pain associated with Raynaud's phenomenon (69%), back pain (47.6%), headache (31%), chest pain (23.8%), odynophagia (21.4%) and painful digital ulcers (19%). Symptoms of neuropathic pain were noticed in 26.2% of patients. Severe joint pain, everyday pain and symptoms of neuropathic pain in SSc were associated with more severe disease and poorer quality of life. Pain related to Raynaud's phenomenon, digital ulcers, odynophagia and joint pain were associated with significant symptoms of depression., Conclusion: The majority of patients with SSc suffer from different types of pain. Pain is associated with more severe disease, depression and poor quality of life., (Copyright © 2019 American Society for Pain Management Nursing. Published by Elsevier Inc. All rights reserved.)

Objectives: The Scleroderma Health Assessment Questionnaire (SHAQ) is a functional scale which consists of five scleroderma-specific items (overall disease severity, Raynaud's phenomenon, digital ulcers, respiratory and intestinal involvement) in addition to Health Assessment Questionnaire Disability Index (HAQ-DI). The objective of this study was to perform an adaptation and validation of a Turkish version of the SHAQ., Method: We validated psychometric properties of the scale with 70 consecutive systemic sclerosis (SSc) patients, who fulfilled the 2013 ACR/EULAR classification criteria for SSc. We evaluated test-retest reliability with the intraclass correlation coefficient (ICC), discriminant validity by stratifying patients according to organ involvements and disease subtypes, and convergent validity by testing the correlation between SHAQ and related components of Short Form 36 version 2 (SF-36v2). Internal consistency of the questionnaire was evaluated by Cronbach's alpha coefficient., Results: The SHAQ-global, visual analogue scales (VAS) of pulmonary, digital ulcer, and Raynaud's phenomenon were significantly correlated with the physical component score of the SF-36v2 (r = - 0.274, r = - 0.295, r = - 0.326, r = - 0.308, p < 0.05, respectively) for the convergent validity. The instruments could not discriminate between disease subtypes, except the digital ulcer VAS which was significantly higher in patients with dcSSc (1.00 ± 0.93 vs 0.55 ± 0.88, p = 0.026) for the discriminant validity. The HAQ-DI, SHAQ-global, digital ulcer VAS, and pulmonary VAS showed moderate correlation with an increase in the number of the organs involved (r = 0.319, r = 0.329, r = 0.341, r = 0.278, p < 0.05, respectively). We demonstrated high reproducibility for HAQ-DI (ICC = 0.962, 95% confidence interval = 0.934-0.978) and the other items of SHAQ. The overall internal consistency of the SHAQ was satisfactory (Cronbach's alpha = 0.953)., Conclusions: The Turkish version of the SHAQ met the requirements of validity and reproducibility.

Objectives: PROMIS-29 is a generic health-related quality of life instrument. Our objective was to assess the reliability, construct validity, and responsiveness to change of PROMIS-29 in systemic sclerosis-associated interstitial lung disease (SSc-ILD)., Methods: Seventy-three participants with SSc-ILD were administered patient reported outcomes (PROs) at baseline and follow-up visits which included PROMIS-29 and other measures of generic health, dyspnea, and cough instruments. We assessed internal consistency reliability using Cronbach's α, an alpha of ≥ 0.70 was considered satisfactory. We assessed the responsiveness to change using linear regression models., Results: Mean age of the participants was 51.9 years and the mean disease duration was 7.9 years after first non-Raynaud's symptom. Of the 73 participants, 56.2% were classified as diffuse SSc and 26% limited SSc. The baseline (mean ± SD) FVC % predicted was 73.9±15.5 with a DLCO % predicted of 57.7±21.1; 95.9% had fibrotic NSIP pattern on HRCT. PROMIS-29 scores were 0.2 to 0.9 SD below the US population. Cronbach's α reliability was acceptable for all domains (ranged from 0.77 to 0.98). All scales showed statistically significant correlations with hypothesised PROMIS-29 domains (p≤0.05 for all comparisons). PROMIS-29 showed none-to-small discriminatory ability in comparison with physiologic measures (FVC and DLCO). There was no significant relationship between the change in FVC versus the change in PROMIS-29 measures over time., Conclusions: PROMIS-29 has adequate reliability and construct validity for evaluation in SSc-ILD. It has moderate-to-large correlations with other PROs. The PROMIS-29 domains were not found to change over time in this cohort, likely due to stable nature of the observational cohort.

Objective: Assessment of cerebrovascular hemodynamics, third ventricle diameter (as a proxy of brain atrophy) by transcranial sonography (TCS), and screening of cognitive performance by the Symbol Digit Modalities Test (SDMT) in systemic sclerosis (SSc) patients., Methods: A total of 38 SSc patients recruited from the outpatient clinic of the Rheumatology Department, Kasr Alainy Hospital, Cairo University, and 51, age- and sex-matched, healthy controls were included in the study. TCS was used to assess the mean flow velocity (MFV), pulsatility index (PI) of the anterior, middle, and posterior cerebral arteries bilaterally, and to measure the third ventricle diameter as a proxy of brain atrophy. Cognitive impairment was screened using the SDMT. p values < 0.05 were considered statistically significant., Results: There was no significant difference between SSc patients and controls regarding either PI or MFV of the anterior, middle, and posterior cerebral arteries; also, there was no difference regarding the third ventricle diameter; however, limited SSc patients showed a significant increase in the PI of PCA and MFV of ACA as compared with diffuse SSc patients (p = 0.005, 0.004). There was a significant difference between SSc patients and controls regarding the SDMT (p = 0.016)., Conclusion: There is an evidence of increased cerebral vascular tone and resistance in limited SSc patients compared with diffuse SSc subgroup, without evidence of cerebral atrophy, suggesting early cerebrovascular affection even in asymptomatic limited SSc patients. There was also an evidence of cognitive impairment in SSc patients.

Purpose: The objectives were to identify reasons why patients attend scleroderma support groups and to ascertain preferences for how meetings are best organized., Methods: The survey included 30-items on reasons for attending and nine items on organizational preferences. Patients were recruited through European patient organizations. Exploratory factor analysis was used to group reasons for attendance thematically., Results: About 213 scleroderma patients (192 women) completed the survey. A three-factor model best described reasons for attending [χ 2 (348) = 586.1, p < 0.001; Comparative Fit Index = 0.98; Tucker Lewis Index = 0.97; Root Mean Square Error of Approximation = 0.06] with themes that included: (1) obtaining interpersonal and social support, (2) learning about treatment and symptom management strategies, and (3) discussing other aspects of scleroderma. Among organizational preferences, respondents emphasized that meetings should include educational aspects and the opportunity to share information and support., Conclusion: People with scleroderma attend support groups to give and obtain social support and for education about managing their disease and other aspects of living with scleroderma. Support groups should be structured to facilitate both educational and informational aspects and to provide opportunities for sharing and support between members. Implications for rehabilitation Local peer-led support groups are an important support and informational resource for patients living with scleroderma. People with scleroderma attend support groups in order to: (1) obtain interpersonal and social support, (2) learn about disease treatment and symptom management strategies, and (3) discuss other aspects of living with scleroderma outside of symptom management. Most support group members prefer groups with a trained facilitator, that include family members or loved ones in the groups, that include between 11and 20 members, that last between 1 and 2 h, and that meet once every 1-3 months. Rehabilitation professionals can support the formation and management of local support groups or can refer patients to national scleroderma patient organizations for information on support groups that they may be able to access.

Digital ulcers are common in patients with systemic sclerosis, affecting over half of patients during the course of their disease. For some patients, digital ulcers occur as isolated phenomena; whereas, for others, digital ulceration is recurrent, and often refractory to intervention. Demonstrating treatment efficacy for digital ulcer disease has typically focussed on clinician opinion of ulcer healing and new ulcer occurrence. Advances in management have improved outcomes which may have had the unfortunate effect of rendering traditional trial endpoints less effective at demonstrating treatment efficacy. Despite recent improvements in management, our work is not complete and digital ulceration remains a major cause of morbidity for many patients with systemic sclerosis. This review shall examine the patient experience of digital ulcers in systemic sclerosis. We shall consider how a detailed understanding of the severity and burden of digital ulceration, aetiopathogenesis, and their impact on emotional health, function, work and social participation might inform the development of novel clinical trial outcomes that can support future advances in the assessment and management of digital ulceration in systemic sclerosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Treatment adherence is an important medical and pharmaco-economical phenomenon, influenced by multiple variables. Treatment adherence in systemic sclerosis (SSc) has been poorly studied., Objective: The aim of the present study was to assess treatment adherence in SSc patients and to identify factors associated with good and poor adherence., Methods: We conducted a monocentric, cross-sectional, observational study. Treatment adherence was evaluated by the Compliance Questionnaire of Rheumatology (CQR). The necessity of treatment and concerns about treatment were investigated using the Beliefs about Medicines Questionnaire-Specific (BMQ-S). The Illness Perception Questionnaire-Revised (IPQ-R) assessed illness perceptions. Disease-related characteristics were collected retrospectively., Results: A total of 66 patients were enrolled in this study. Of these, 47 (71.2%) had a weighted CQR score of ≤80% ("poor adherence") and 19 (28.8%) had a weighted CQR score of >80% ("good adherence"). No significant relationship between demographic, clinical or psychological factors and overall adherence could be found, except with the IPQ subscale "timeline acute/chronic" (p = 0.042). Our patient population estimated the necessity of their medication high (mean necessity score 20.5), with moderate concern beliefs (mean concern score 15.1). Subjective adherence, as self-reported by patients, was high., Conclusions: This study demonstrated low treatment adherence rates in SSc patients. We could not identify demographic, clinical or psychological factors associated with treatment adherence, except with the IPQ subscale "timeline acute/chronic". This suggests a correlation between poor adherence and the belief that the disease will be chronic without improvement over time. Symptom relief was an important motivating factor for taking medication. The treatment necessity was scored higher than treatment concerns, but the necessity beliefs were not associated with adherence., (© 2018 John Wiley & Sons, Ltd.)

Objective: In a pilot study, our group showed that an internet-based self-management program improves self-efficacy in systemic sclerosis (SSc). The objective of the current study was to compare an internet-based self-management program to a patient-focused educational book developed to assess measures of self-efficacy and other patient-reported outcomes in patients with SSc., Methods: We conducted a 16-week randomized, controlled trial., Results: Of the 267 participants who completed baseline questionnaires and were randomized to the intervention (internet: www.selfmanagescleroderma.com) or control (book) group, 123 participants (93%) in the internet group and 124 participants (94%) in the control group completed the 16-week randomized controlled trial (RCT). The mean ± SD age of all participants was 53.7 ± 11.7 years, 91% were women, and 79.4% had some college or a higher degree. The mean ± SD disease duration after diagnosis of SSc was 8.97 ± 8.50 years. There were no statistical differences between the 2 groups for the primary outcome measure (Patient-Reported Outcomes Measurement Information System Self-Efficacy for Managing Symptoms: mean change of 0.35 in the internet group versus 0.94 in the control group; P = 0.47) and secondary outcome measures, except the EuroQol 5-domain instrument visual analog scale score (P = 0.05). Internet group participants agreed that the self-management modules were of importance to them, the information was presented clearly, and the website was easy to use and at an appropriate reading level., Conclusion: Our RCT showed that the internet-based self-management website was not statistically superior to an educational patient-focused book in improving self-efficacy and other measures. The participants were enthusiastic about the content and presentation of the self-management website., (© 2018, American College of Rheumatology.)

To assess clinical and psychosocial factors related to alexithymia in systemic sclerosis (SSc). We enrolled 40 consecutive SSc patients in a cross-sectional study evaluating alexithymia with Toronto Alexithymia scale (TAS-20). We measured Beck Depression inventory (BDI), Hamilton Anxiety rating scale (HAM-H), 36-Items Short-Form Healthy Survey (SF-36), Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue, Visual Analog Scale (VAS) pain, Pittsburgh Sleep Quality Index (PSQI), Satisfaction with Appearance Scale (SWAP), and Mouth Handicap in Systemic Sclerosis (MHISS). The prevalence of alexithymia was 42%. Alexithymic patients presented increased depressive (p = ≤ 0.001) and anxiety symptoms (p = ≤ 0.001), sleep disorders (p = 0.03), pain (p = 0.02), esthetic concerns (p = 0.03), disability in activities (p = 0.03) and reduced scores of SF-36 in mental components summary (MCS) (p = ≤ 0.001) and physical components summary (PCS) (p = 0.01). We found significant correlations with sleep disorders (r = 0.41, p = ≤ 0.001), BID (r = 0.35, p = 0.04), facial image dissatisfaction (r = 0.35, p = 0.04), mouth disability (r = 0.51, p = 0.005), depressive (r = 0.6, p = ≤ 0.001), and anxiety symptoms (r = 0.48, p = ≤ 0.001), fatigue (r = - 0.45 p = 0.005), SF-36 PCS (r = - 0.51, p = ≤ 0.001) and MCS (r = - 0.65, p = ≤ 0.001). In multiple linear regression analysis, SWAP facial was the only variable associated with TAS-20 [0.99 (0.48) p = 0.05]. Alexithymia correlates with several psychosocial factors but seems strongly related to facial image dissatisfaction.

Objectives: The 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 to develop and validate a short-form SAAS based on objective and reproducible criteria., Design: This study was a cross-sectional analysis of baseline data from adults enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort., Setting: Adults in the SPIN Cohort in the present study were enrolled at 28 centres in Canada, the USA and the UK., Participants: The SAAS was administered to 926 adults with scleroderma., Primary and Secondary Measures: The SAAS, Brief Fear of Negative Evaluation II (BFNE II), Brief Satisfaction with Appearance Scale (Brief-SWAP), Patient Health Questionnaire-8 (PHQ8) and Social Interaction Anxiety Scale-6 (SIAS-6) were collected, as well as demographic characteristics., Results: OTA methods identified a maximally informative shortened version for each possible form length between 1 and 15 items. The final shortened version was selected based on prespecified criteria for reliability, concurrent validity and statistically equivalent convergent validity with the BFNE II scale. A five-item short version was selected (SAAS-5). The SAAS-5 had a Cronbach's α of 0.95 and had high concurrent validity with the full-length form (r=0.97). The correlation of the SAAS-5 with the BFNE II was 0.66, which was statistically equivalent to that of the full-length form. Furthermore, the correlation of the SAAS-5 with the two subscales of the Brief-SWAP, and the SIAS-6, were statistically equivalent to that of the full-length form., Conclusions: OTA was an efficient method for shortening the full-length SAAS to create the SAAS-5., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of the skin and the involvement of multiple internal organs. Previous studies reported poorer health-related quality of life (HRQoL) in patients with SSc compared with the general population. However, very little is known about how HRQoL in SSc patients compares with that in patients with other systemic autoimmune diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjogren's syndrome (SjS). Thus, the main aim of this study was to compare HRQoL in SSc patients, patients with other rheumatic diseases, and the general population., Methods: In this cross-sectional study, patients from the rheumatology clinics of Seoul National University Hospital with SSc, RA, SLE, and SjS were enrolled via a random sampling technique. HRQoL was captured by the Short Form (36) health survey (SF-36), the Short Form Six-Dimensional health index (SF-6D), and the EuroQol Five-Dimensional descriptive system (EQ-5D). Demographic characteristics and standardized disease activity for each disease were also obtained. Previously reported data from 600 healthy Koreans were used for the healthy controls. An ANCOVA test was used to compare the SF-36, SF-6D, and EQ-5D values between study subjects with adjustments for age, sex, disease duration, comorbidities, and disease activity status., Results: One hundred twenty patients were included in each of the SSc, RA, SLE, and SjS cohorts. Patients with rheumatic diseases had significantly lower SF-36, SF-6D, and EQ-5D scores than healthy controls (all P < 0.001). After statistical adjustments, SSc patients reported significantly lower mental component summary (MCS) scores than patients with RA (P < 0.001) or SLE (P = 0.001). Specifically, the mental health and general health domains were significantly lower in SSc patients than reported in RA or SLE patients (P < 0.001 and P = 0.001, respectively, in both domains). In SSc patients, higher modified Rodnan skin scores (mRSS) correlated with lower MCS scores., Conclusions: SSc patients report poorer HRQoL than patients with RA or SLE. The extent of skin involvement is associated with poorer HRQoL in SSc patients., Trial Registration: NCT03257878 . Registered 22 August 2017.

Objective: To investigate clinical manifestations of juvenile systemic sclerosis (SSc; scleroderma), including disease characteristics and patient quality of life, using the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry., Methods: Patients with juvenile SSc were prospectively enrolled between 2010 and 2013. The diagnosis of juvenile SSc was determined by the enrolling pediatric rheumatologist, with the requirement for disease onset prior to age 18 years. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics., Results: In total, 64 patients with juvenile SSc were enrolled a median of 3.6 years after disease onset, which occurred at a median age of 10.3 years. The most common organ manifestations were dermatologic and vascular, followed by musculoskeletal, gastrointestinal, and pulmonary; in 38% of patients, ≥4 organ systems were affected. Patients with juvenile SSc had significantly more disability at enrollment compared with CARRA Legacy Registry patients with juvenile idiopathic arthritis, dermatomyositis, or systemic lupus erythematosus. Although physician-reported measures correlated most closely with arthritis, dermatologic manifestations, and pulmonary manifestations, poor patient-reported measures were associated with gastrointestinal involvement. During >50 person-years of follow-up, most organ manifestations remained stable, and no mortality or development of new solid organ involvement after enrollment was reported., Conclusion: In the first multicenter prospective cohort of patients with juvenile SSc in North America, the disease burden was high: multiorgan manifestations were common, and functional disability was greater than that observed in patients with other childhood-onset rheumatic diseases. Gastrointestinal involvement had the greatest impact on quality of life., (© 2018, American College of Rheumatology.)