Quality of sleep in individuals with systemic sclerosis and its correlation with functional disability and quality of life: a cross-sectional study.

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.

Severity and impact of digestive impairment perceived by patients with systemic sclerosis: a cross-sectional study.

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 (ß=0.249; p=0.002) and Visual Analogue Scale 3-Level EuroQol-5D (VAS-EQ-5D-3L) (ß=-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.

The association of resilience and positive mental health in systemic sclerosis: A Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study.

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.

Moderators of Loneliness Trajectories in People with Systemic Sclerosis During the COVID-19 Pandemic: A SPIN COVID-19 Cohort Longitudinal Study.

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.

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

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.

Systemic Sclerosis Quality of Life Questionnaire (SScQoL): translation into Turkish and assessing its psychometric properties.

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.

Mental health symptoms in scleroderma during COVID-19: a Scleroderma Patient-centred Intervention Network (SPIN) cohort longitudinal study.

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.

Perceived Cognitive Function in People With Systemic Sclerosis: Associations With Symptoms and Daily Life Functioning.

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.

Cognitive difficulties in people with systemic sclerosis: a qualitative study.

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 relationship of ocular parameters with clinical parameters and disease-related quality of life in patients with systemic sclerosis: A cross-sectional study.

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.

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

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.

Happiness, quality of life and their determinants among people with systemic sclerosis: a structural equation modelling approach.

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 (ß = -0.79, P < 0.001) and with happiness (ß = -0.52, P < 0.001). Positive personality traits had a positive relation with happiness (ß = 0.36, P = 0.002) and an important indirect association upon QoL (ß = 0.43) and happiness (ß = 0.23). Perceived disease impact is influenced by body image, fatigue and SSc-related disability to a higher degree (ß = 0.6-0.7) than by disease activity (ß = 0.28) or form (ß = 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.

Unmet Workplace Support Needs and Lost Productivity of Workers With Systemic Sclerosis: A Path Analysis Study.

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.

Fatigue and Its Association With Social Participation, Functioning, and Quality of Life in Systemic Sclerosis.

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 (R2 = 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.

The Relationship Between Autonomic Dysfunction of the Gastrointestinal Tract and Emotional Distress in Patients With Systemic Sclerosis.

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.

COVID-19 and systemic sclerosis: analysis of lifestyle changes during the SARS-CoV-2 pandemic in an Italian single-center cohort.

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.

Validation of the COVID-19 Fears Questionnaires for Chronic Medical Conditions: A Scleroderma Patient-centered Intervention Network COVID-19 Cohort study.

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 (χ2(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.

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

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.