Increased Confidence and Deeper Understanding of Fatigue Following Participation in Fatigue Education and Management Education in Systemic Sclerosis: A Mixed Methods Evaluation of a Virtual Intervention.

OBJECTIVE: No fatigue-specific programs exist for people with systemic sclerosis (SSc) despite the burden of fatigue and negative impact on daily activities. This study used a convergent parallel mixed methods design to evaluate the impact of an adapted virtual intervention, Fatigue and Activity Management Education in Systemic Sclerosis (FAME-iSS), in the United States. METHODS: Eighteen people with SSc participated in three separate six-week FAME-iSS programs. Participants completed the modified Fatigue Impact Scale (m-FIS), the Self-Efficacy for Performing Energy Conservation Strategies Assessment (SEPESCA), the Patient-Reported Outcomes Measurement Information System (PROMIS) Self-Efficacy for Managing Symptoms, and the Hospital Anxiety and Depression Scale (HADS) before, immediately after, and three months post intervention. Data were analyzed using descriptive and nonparametric inferential statistics. Participants' perceptions of the program and their use of fatigue management strategies were qualitatively analyzed using content analysis. RESULTS: Eighty-nine percent of participants were women with a mean ± SD age of 52.0 ± 11.6 years and a mean ± SD disease duration of 13.7 ± 14.5 years, and more than 70% had a college degree. Significant improvements were observed for self-efficacy on the PROMIS Self-Efficacy for Managing Symptoms (P = 0.002) and SEPESCA (P = 0.016) immediately post intervention, which continued to significantly improve up to the three-month follow-up (P = 0.006 and 0.035, respectively). Significant improvements were also observed for the m-FIS between baseline and the three-month follow-up (P = 0.029). Participants reported a deeper understanding of fatigue and that they liked sharing strategies and experiences with each other along with the facilitator, citing that "there was a power in our group because we had a common condition." CONCLUSION: FAME-iSS resulted in improvements in the impact of fatigue and self-efficacy for managing symptoms and performing energy conservation strategies. Feedback was positive, and the virtual format allowed for greater accessibility and sharing of strategies.

EULAR recommendations for the non-pharmacological management of systemic lupus erythematosus and systemic sclerosis.

OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological management of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). METHODS: A task force comprising 7 rheumatologists, 15 other healthcare professionals and 3 patients was established. Following a systematic literature review performed to inform the recommendations, statements were formulated, discussed during online meetings and graded based on risk of bias assessment, level of evidence (LoE) and strength of recommendation (SoR; scale A-D, A comprising consistent LoE 1 studies, D comprising LoE 4 or inconsistent studies), following the European Alliance of Associations for Rheumatology standard operating procedure. Level of agreement (LoA; scale 0-10, 0 denoting complete disagreement, 10 denoting complete agreement) was determined for each statement through online voting. RESULTS: Four overarching principles and 12 recommendations were developed. These concerned common and disease-specific aspects of non-pharmacological management. SoR ranged from A to D. The mean LoA with the overarching principles and recommendations ranged from 8.4 to 9.7. Briefly, non-pharmacological management of SLE and SSc should be tailored, person-centred and participatory. It is not intended to preclude but rather complement pharmacotherapy. Patients should be offered education and support for physical exercise, smoking cessation and avoidance of cold exposure. Photoprotection and psychosocial interventions are important for SLE patients, while mouth and hand exercises are important in SSc. CONCLUSIONS: The recommendations will guide healthcare professionals and patients towards a holistic and personalised management of SLE and SSc. Research and educational agendas were developed to address needs towards a higher evidence level, enhancement of clinician-patient communication and improved outcomes.

Fatigue as an Understudied Barrier to Participation in Life Roles.

Fatigue is one of the most burdensome and disabling symptoms in numerous acute and chronic conditions and is associated with reduced participation in all aspects of daily life, for example, parenting, employment, and socialization. Historically, occupational practitioners played key roles in fatigue management by creating and implementing interventions. The American Occupational Therapy Foundation convened a Planning Grant Collective workshop with the goal to develop collaborative research ideas and proposals to advance the understanding and management of fatigue. Participants from occupational therapy and other disciplines, with expertise with fatigue or who were conducting research on fatigue, participated in a 3-day virtual workshop. Four main topics emerged: implementation science, treatment taxonomy, trial design and comparative effectiveness, and phenotyping. This white paper describes the proceedings, summarizes the discussions, and outlines recommendations from the PGC workshop on fatigue.

2022 American College of Rheumatology Guideline for Exercise, Rehabilitation, Diet, and Additional Integrative Interventions for Rheumatoid Arthritis.

OBJECTIVE: To develop initial American College of Rheumatology (ACR) guidelines on the use of exercise, rehabilitation, diet, and additional interventions in conjunction with disease-modifying antirheumatic drugs (DMARDs) as part of an integrative management approach for people with rheumatoid arthritis (RA). METHODS: An interprofessional guideline development group constructed clinically relevant Population, Intervention, Comparator, and Outcome (PICO) questions. A literature review team then completed a systematic literature review and applied the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach to rate the certainty of evidence. An interprofessional Voting Panel (n = 20 participants) that included 3 individuals with RA achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations. RESULTS: The Voting Panel achieved consensus on 28 recommendations for the use of integrative interventions in conjunction with DMARDs for the management of RA. Consistent engagement in exercise received a strong recommendation. Of 27 conditional recommendations, 4 pertained to exercise, 13 to rehabilitation, 3 to diet, and 7 to additional integrative interventions. These recommendations are specific to RA management, recognizing that other medical indications and general health benefits may exist for many of these interventions. CONCLUSION: This guideline provides initial ACR recommendations on integrative interventions for the management of RA to accompany DMARD treatments. The broad range of interventions included in these recommendations illustrates the importance of an interprofessional, team-based approach to RA management. The conditional nature of most recommendations requires clinicians to engage persons with RA in shared decision-making when applying these recommendations.

2022 American College of Rheumatology Guideline for Exercise, Rehabilitation, Diet, and Additional Integrative Interventions for Rheumatoid Arthritis.

OBJECTIVE: To develop initial American College of Rheumatology (ACR) guidelines on the use of exercise, rehabilitation, diet, and additional interventions in conjunction with disease-modifying antirheumatic drugs (DMARDs) as part of an integrative management approach for people with rheumatoid arthritis (RA). METHODS: An interprofessional guideline development group constructed clinically relevant Population, Intervention, Comparator, and Outcome (PICO) questions. A literature review team then completed a systematic literature review and applied the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach to rate the certainty of evidence. An interprofessional Voting Panel (n = 20 participants) that included 3 individuals with RA achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations. RESULTS: The Voting Panel achieved consensus on 28 recommendations for the use of integrative interventions in conjunction with DMARDs for the management of RA. Consistent engagement in exercise received a strong recommendation. Of 27 conditional recommendations, 4 pertained to exercise, 13 to rehabilitation, 3 to diet, and 7 to additional integrative interventions. These recommendations are specific to RA management, recognizing that other medical indications and general health benefits may exist for many of these interventions. CONCLUSION: This guideline provides initial ACR recommendations on integrative interventions for the management of RA to accompany DMARD treatments. The broad range of interventions included in these recommendations illustrates the importance of an interprofessional, team-based approach to RA management. The conditional nature of most recommendations requires clinicians to engage persons with RA in shared decision-making when applying these recommendations.

Expanding the Treatment Team: What Is the Role for Occupational Therapy, Physical Therapy, Wound Care, and Nutritional Support in Systemic Sclerosis?

The optimal systemic sclerosis (SSc) care plan includes an occupational therapist and physical therapist as well as wound care experts and a registered dietitian if indicated. Screening instruments for functional and work disability, hand and mouth limitations, malnutrition, and dietary intake can identify the need for ancillary support services. Telemedicine can assist in developing effective ancillary treatment plans. Reimbursement for services may limit access for patients with SSc to expand their care team but a focus on prevention rather than management of damage is recognized as an important unmet need in SSc. In this review, the role of a comprehensive care team for SSc is discussed.

'It's just not the same I am just a spectator': A qualitative study on changes in leisure participation experienced by people with scleroderma.

BACKGROUND/OBJECTIVE: The purpose of this qualitative study was to provide insight into the changes and barriers to leisure experienced by people with scleroderma. METHOD: Twenty-five people with scleroderma consented to be interviewed via telephone using a semi-structured interview. The conversations were audio recorded and transcribed verbatim. Thorne's Interpretive Description informed the analysis process. RESULTS: Three themes emerged from the analysis: barriers to leisure participation (impact of Raynaud's Phenomenon symptoms, fatigue, changes in body structures and functions, unpredictability of daily symptoms and selection of leisure activities), decreases in leisure participation (less time outdoors, reduction in time spent in active leisure, more time spent in passive leisure) and experiences of losing a valued leisure activity (depressed mood, identity change, fear of loss and sense of isolation). CONCLUSIONS: The changes in leisure participation found in this study were reported to be due to the rate of disease progression and the severity of disease symptoms, particularly, Raynaud symptoms and fatigue. Health professionals should work with people with SSc to facilitate participation in valued leisure activities.

Perspective of Patients and Stakeholders as Members of a Research Team.

Systemic sclerosis (SSc) is a rare orphan disease, characterized by skin thickening, vascular insufficiency, and fibrosis of internal organs. SSc affects about 100,000 people in the United States. This study explored perceived benefits and challenges of patient partners and stakeholders, who were team members on a project to revise and test a self-management program (Taking Charge of Systemic Sclerosis). Five patient partners, 1 stakeholder from the Scleroderma Foundation and 1 stakeholder from a state chapter of the Scleroderma Foundation were interviewed. Conversations were audio recorded and transcribed verbatim and analyzed. Four themes emerged from the analysis with corresponding subthemes: contributions to study, benefits of involvement, challenges, and project leadership. The themes and subthemes were generally similar to those expressed in other studies. However, additional benefits from engagement were identified: acceptance, increased knowledge of SSc, and helping others. Participants reported feeling supported and valued as members of the team and that their opinions mattered which is in contrast with findings from other studies.

Importance and difficulty with valued life activities for people with systemic sclerosis.

PURPOSE: To identify the importance of and difficulty with valued activities in persons with systemic sclerosis (SSc) and to examine relationships between disease symptoms and difficulty with valued activities using the Valued Activities Scale (VLA). A secondary purpose was to examine the internal consistency of the Short-VLA Scale (S-VLA). METHODS: A cross-sectional convenience sample of 99 people with SSc completed questionnaires regarding demographics, symptom severity, Health Assessment Questionnaire (HAQ), Center for Epidemiologic Studies Depression Scale (CES-D), and the VLA. RESULTS: Obligatory activities were rated as most important; committed activities were significantly more difficult than obligatory and discretionary. Less fatigue (p < 0.01) and lower HAQ (p < 0.001) and CES-D (p < 0.01) scores were associated with higher total VLA scores. Internal consistency of the S-VLA was excellent (Cronbach's alpha = 0.92; p < 0.0001). The correlation between the S-VLA and the VLA was excellent (r = 0.96; p < 0.001). There were moderate correlations between the S-VLA and the HAQ (r = 0.73; p < 0.0001); the correlation with the CES-D was only fair (r = 0.35; p < 0.001). CONCLUSIONS: Committed and discretionary activities were more difficult for people with SSc to perform. Results are similar to findings with people with rheumatoid arthritis and systemic lupus erythematosus.IMPLICATIONS FOR REHABILITATIONHome management/caregiving (committed) and leisure and social participation (discretionary) activities are more difficult to perform by people with SSc compared to self-care (obligatory) activities.Our findings that difficulty scores on the VLA were associated with more fatigue and depression suggest the need for rehabilitation to reduce disability in people with SSc.The S-VLA may be a useful screening and monitoring tool for SSc and other chronic conditions.

World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease.

Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF. Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included. Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94-100% for the three additional PROMs. Thirty-four new 'Personal Factors' emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. 'pf_embarrassed by cough' or 'pf_panic/afraid when can't get a breath'. Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11.

Beyond the hand and upper extremity: The role of hand therapists in care of people with rheumatic diseases.

STUDY DESIGN: Invited Clinical Commentary BACKGROUND: Arthritis is one of the most frequently reported causes of disability in the United States and the prevalence is expected to increase in the coming decades. While many rheumatic diseases involve hand impairments, most are systemic and involve more than the musculoskeletal system. Functional and work disability are high and people would benefit from the services of occupational and physical therapists. PURPOSE OF STUDY: This paper reviews concepts of self-management, and symptoms that contribute to limitations and restrictions to participation in daily life in people with rheumatic diseases and suggests roles for hand therapists beyond the immediate hand impairments. METHODS: The impact of selected rheumatic diseases on functional and work disability are reviewed along with strategies for symptom management and self-management. Upper extremity impairments of selected rheumatic diseases are also discussed. RESULTS: The role for hand therapists in evaluating and addressing the complex needs of persons with rheumatic diseases, including less common diseases, is discussed. Outcome measures for fatigue, muscle involvement, ergonomics and computer use, and work disability are introduced. Finally, strategies for self-management and prevention of work and functional disability, along with symptom management for fatigue and pain are presented. CONCLUSION: Hand therapists can play a vital role in chronic rheumatic disease management to improve self-management and increase participation in meaningful activities. Patients, primary care and rheumatology providers need to be educated about the scope of services occupational and physical therapists provide beyond the hand impairments.

Systemic sclerosis in Native Americans of the American Southwest.

OBJECTIVE: Many indigenous non-Caucasian populations, including Native Americans, have been reported to have higher rates, distinct clinical phenotypes, increased complications, and greater severity of systemic sclerosis (SSc). However, little is known of SSc specifically in Native Americans of the American Southwest. This study compared the clinical and serologic manifestations and outcomes of SSc in Native Americans and non-Native Americans (non-Natives) of this region. METHODS: This cross-sectional retrospective study included 137 SSc patients (109 [80%] were non-Native and 28 [20%] were Native Americans) followed over a mean of 11.5 ± 7.6 years. Participants were repetitively evaluated with medical history, physical examination, echocardiography, chest imaging, and serologic testing. Disease characteristics and outcomes were statistically compared between Native Americans and non-Native patients. RESULTS: The estimated prevalence of SSc in Native Americans was 40.0 cases/100 000 vs 17.1 cases/100 000 for non-Natives (odds ratio 2.34, 95% confidence interval [CI] 1.55-3.55, P < .001). The cohorts were similar in terms age, age of onset, limited vs diffuse cutaneous SSc, telangiectasias, gastroesophageal reflux disease, Raynaud phenomenon, serologies, interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, cancer prevalence, and overall mortality (all P > .05). However, for Native Americans, mortality specifically from fatal infections was 3.94-fold that of non-Natives (hazard ratio 6.88, 95% CI 1.37-34.64; P < .001). CONCLUSION: In Native Americans of the American Southwest, SSc is increased in prevalence but is phenotypically similar to SSc in non-Natives. However, mortality due specifically to infection is increased in Native Americans with SSc.

Reliability, validity, and responsiveness to change of the Patient-Reported Outcomes Measurement Information System self-efficacy for managing chronic conditions measure in systemic sclerosis.

Objective: The aim of this study is to examine validity, reliability, and responsiveness to change of Patient-Reported Outcomes Measurement Information System Self-Efficacy for Managing Chronic Conditions in persons with systemic sclerosis. Methods: We conducted a post hoc analysis of the Patient-Reported Outcomes Measurement Information System Self-Efficacy measure and other quality-of-life measures from systemic sclerosis participants from a 16-week randomized control trial. The trial compared an Internet-based self-management program to a control condition where participants were provided an educational book. All participants completed outcome measures at baseline and following the 16-week trial period. Results: The mean age of participants was 53.7 years, 91% were female and systemic sclerosis subtype included 44.9% limited/sine and 43.1% diffuse; mean disease duration was 9.0 years. All self-efficacy subscales (Managing Emotions, Symptoms, Daily Activities, Social Interactions, and Medications/Treatment) demonstrated good internal consistency (.92-.96). All subscales showed statistically significant correlations with other validated measures of depressive symptoms and quality of life (.20-.86) but were not associated with satisfaction nor with appearance. The subscales appropriately discriminated between those with and without depressive symptoms and demonstrated responsiveness to change over the 16-week period for those who had a corresponding increase in reported quality of life. Conclusion: The Patient-Reported Outcomes Measurement Information System Self-Efficacy measure is valid, reliable, and responsive to change for persons with systemic sclerosis.

Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest.

Objective: Certain Hispanic/Latino (Hispanic) populations have been reported to have higher rates and severity of systemic sclerosis; however, little is known of systemic sclerosis in the American Southwest. This study compared manifestations of systemic sclerosis in Hispanics with non-Hispanics of New Mexico. Methods: This cross-sectional longitudinal study included 109 systemic sclerosis patients followed over a mean of 12.6 ± 8.9 years. Subjects were repetitively evaluated including physical examination, echocardiography, chest imaging, and serologic testing and observed for complications. Disease characteristics and long-term outcomes were statistically compared between self-identified Hispanic and non-Hispanic subjects. Results: A total of 73 (67%) systemic sclerosis subjects were Hispanic and 36 (33%) were non-Hispanic. The cohorts were similar in mean age, age of systemic sclerosis onset, limited versus diffuse cutaneous systemic sclerosis, telangiectases, gastroesophageal reflux disease, Raynaud's phenomenon, autoantibody profile, interstitial lung disease, pulmonary hypertension, scleroderma renal crisis, mortality, and comorbid malignancy (all p > 0.05). However, the standardized mortality ratio was increased in both cohorts relative to age-adjusted mortality: Hispanic: 2.08, confidence interval (1.94-2.24); non-Hispanic: 1.56, confidence interval (1.46-1.68). Furthermore, the standardized incidence ratio for malignancy was increased in both cohorts: Hispanic: 1.45, confidence interval (1.35-1.56); non-Hispanic: 1.24, confidence interval (1.16-1.34). The mean age of cancer diagnosis occurred at a significantly younger age in Hispanics (Hispanics: 53.1 ± 9.7 years; non-Hispanics 63.7 ± 7.9 years; 95% confidence interval: -19 ⩽ 10.6 ⩽ 2.2; p = 0.016). Conclusion: Systemic sclerosis phenotype, autoantibodies, complications, outcomes, malignancy rates, and mortality are generally similar between Hispanics and non-Hispanics with systemic sclerosis in the American Southwest. However, age-adjusted comorbid malignancy and mortality rates are significantly increased in both groups.

The association between hand disease severity and fatigue in individuals with systemic sclerosis: a scoping review.

BACKGROUND: Hand disease severity in people with systemic sclerosis (SSc) arises from connective tissue and vascular changes causing functional limitations, pain, and disability. Fatigue is not well-understood in SSc and reported to be highly distressing. It is not known how fatigue relates to these disease changes. The objective of this study was to identify which elements of hand disease severity contribute to fatigue in individuals with SSc. METHODS: Five online databases and Google Scholar were searched to identify publications through 2021 presenting data related to hand disease severity and fatigue. RESULTS: Five articles met the inclusion criteria. The samples were 72-91% female, and 35-100% with diffuse SSc. Measures of disease severity included skin thickening (modified Rodnan score), joint involvement (mobility, tenderness, swelling), and digital ulcers. Fatigue was measured by various self-report. Joint involvement was strongly associated with fatigue. Skin thickening weakly associated with fatigue. The perceived interference of digital ulcers in daily activities was related to fatigue. CONCLUSIONS: Few studies have examined how fatigue relates to hand disease severity. While joint involvement was associated with fatigue over time and in cross-sectional studies, digital ulcers may be associated with fatigue indirectly. A conceptual model is proposed with implications for future research.Implications for RehabilitationStructural disease changes that manifest in the hands, is often associated with pain, disability, and fatigue in SSc.Fatigue is one of the most distressing symptoms of SSc and reported by 61-90% of individuals with SSc, but is not well characterized.Of measures of hand disease severity, joint involvement (contractures, swollen and tender joints) is most associated with fatigue in individuals with SSc and more so if pain is involved.A better understanding of the physiological features of disease and fatigue could provide insight into development of fatigue management interventions and help rheumatology providers work with patients to manage their fatigue.Rehabilitation assessments and interventions for hand disability need to be considered as important elements in improving all elements of quality of life for individuals with systemic sclerosis.

Rehabilitation Interventions in Systemic Sclerosis: A Systematic Review and Future Directions.

OBJECTIVE: To systematically review evidence of rehabilitation interventions for improving outcomes in systemic sclerosis (SSc) and to evaluate evidence quality. METHODS: Several electronic databases were searched to identify studies in which rehabilitation professionals delivered, supervised, or participated in interventions for individuals with SSc. Randomized controlled trials (RCTs) or non-randomized trials, one-arm trials, and prospective quasi-experimental studies with interventions were included if they had ≥10 participants. Quality appraisal was conducted by 2 independent raters using the Physiotherapy Evidence Database (PEDro) Scale. RESULTS: A total of 16 good or excellent quality studies (15 RCTs, 1 prospective quasi-experimental study) were included. Most rehabilitation interventions focused on hands/upper extremities, followed by multicomponent, orofacial, and directed self-management. Sample sizes varied between 20-267 participants (median 38). In 50% of studies, participants in intervention groups significantly improved compared to controls. Most studies demonstrated within-group improvements in intervention groups. Interventions varied in content, delivery, length, and dose and outcome measures collected. CONCLUSION: Existing evidence provides some support for rehabilitation in SSc, such as interventions that focus on hand and upper extremity outcomes or are multicomponent, although there is high study heterogeneity. The evidence base would benefit from interventions testing similar replicable components, use of common outcome measures, and incorporation of delivery modes that enable larger sample sizes. There are challenges in recruiting participants due to the rarity of SSc and high disease burden, as participants' involvement in rehabilitation studies requires active participation over time. Intervention studies designed to reduce participation barriers may facilitate translation of effective interventions into practice.

Fatigue Predicts Future Reduced Social Participation, not Reduced Physical Function or Quality of Life in People with Systemic Sclerosis.

OBJECTIVE: Although fatigue is one of the most problematic symptoms for people with systemic sclerosis, little is known about how fatigue impacts daily life over time. Such information is important when developing fatigue management interventions. This study was conducted to examine 1) if fatigue severity predicted outcomes of worse functioning (social participation, physical function), and quality of life and 2) if level of self-efficacy moderated significant relationships between fatigue and these outcomes. METHODS: Data were utilized from a clinical trial in which an online self-management intervention was tested (N = 267). Fatigue, social participation, and physical function were assessed by PROMIS measures. Quality of life was assessed by the EuroQol 5-domain instrument (EQ-5D-5L). Linear regressions were performed to examine how baseline fatigue related to functioning and quality of life outcomes 16 weeks later controlling for relevant covariates. PROMIS measures were used to measure self-efficacy in managing symptoms, daily activities, medications and treatments, emotions, and social interactions. RESULTS: Fatigue at baseline significantly predicted social participation 16 weeks later; but did not predict physical functioning or quality of life. Self-efficacy variables did not moderate the association between fatigue and social participation. CONCLUSION: Fatigue severity predicted decreased social participation in people with systemic sclerosis. Interventions targeting fatigue should include support to maintain participation of social roles and activities. The level of reported self-efficacy did not vary the strength of the association between fatigue and decline in social participation indicating that there may be other targets to treat fatigue intervention beyond self-management. TRIAL REGISTRATION: NCT02494401.

Suitability for e-health of non-pharmacological interventions in connective tissue diseases: scoping review with a descriptive analysis.

OBJECTIVE: Non-pharmacological interventions support patients with connective tissue diseases to better cope with and self-manage their diseases. This study aimed to map existing evidence on non-pharmacological interventions in patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and mixed connective tissue diseases regarding content, feasibility and potential suitability in an e-health setting. METHODS: A literature search was performed in eight different databases in July 2020. The intervention's content was extracted using the 'Better reporting of interventions: template for intervention description and replication (TIDieR) checklist and guide'. A Sankey diagram and descriptive statistics were used to analyse the data and illustrate the relationships between the interventions. RESULTS: Of 8198 identified records, 119 papers were eligible. One hundred and four of them (87.4%) were conducted between 2000 and 2020, mainly in the USA (SLE n=24 (21.2%), SSc n=16 (14.2%)), Brazil (SLE n=8 (7.1%), SSc n=5 (4.4%)) and Italy (SLE n=0 (0%), SSc n=12 (10.6%)). Fifty-two studies (SLE n=24 (21.2%), SSc n=28 (24.8%)) used multicomponent interventions. The single interventions were physical exercises (SLE n=16 (14.2%), SSc n=17 (15.0%)), coaching/counselling (SLE n=11 (18.0%), SSc n=0 (0%)) and education (SLE n=2 (1.8%), SSc n=3 (2.7%)). Primary outcomes focused on physical function (SLE n=1 (0.9%), SSc n=15 (13.3%)), mouth opening in SSc (n=4 (5.9%)) and physical capacity (SLE n=2 (1.8%), SSc n=1 (0.9%)). No interventions for mixed connective tissue disease were found. CONCLUSION: There was a great variety in the intervention's content due to differences in body structure, activity limitations and participation restrictions in SLE and SSc. These results highlight the need for personalised, multicomponent, non-pharmacological interventions, which could be delivered as e-health interventions.