Assessment of fertility and sexual dysfunction in women with systemic sclerosis: a narrative review of the literature.

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.

Sexual dysfunction among female patients with rheumatic diseases.

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.

Emotional intelligence: a novel predictor of quality of life in patients with systemic sclerosis.

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.

Association of COVID-19 vaccination and anxiety symptoms: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort longitudinal study.

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.

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.

Use and perceptions of nutrition information resources in systemic sclerosis: a Scleroderma Patient-centred Intervention Network (SPIN) cohort study.

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.

Peer-Led Symptom Management Intervention to Enhance Resilience in People With Systemic Sclerosis: Mediation Analysis From a Randomized Clinical Trial.

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.

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.

Quantifying the Need for Specialist Palliative Care Management in Patients With Systemic Sclerosis.

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.

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.

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.

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.

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.