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.

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.

The evaluation of a home-based program for hands in patients with systemic sclerosis.

STUDY DESIGN: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program. INTRODUCTION: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease. PURPOSE OF THE STUDY: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc. METHODS: A home-based self-management program that consisted of concise instructions about SSc and hand exercises was developed and evaluated in a group of patients with SSc during 8 weeks. Primary outcome measures were hand pain (Visual Analogue Scale) and hand function (Cochin Hand Function Scale). Secondary outcome measures were disability (Scleroderma Health Assessment Questionnaire), finger motion (delta finger-to-palm), grip strength, tip and key pinch strength, Raynaud phenomenon and digital ulcers impact, quality of life (Short Form Health Survey). For comparisons between different times analysis of variance for repeated measures was used. To calculate the effect size (ES), the Cohen's test was performed. To evaluate skin moisturizing and warming habits before and after intervention, the McNemar test was used. Statistical significance was set at P ≤ .05. RESULTS: Twenty-two SSc patients (19 women: 3 men; 16 limited scleroderma: 6 diffuse scleroderma) completed the program. Significant improvements were noted for hand pain (3.97 vs 2.21, ES: 0.69), Cochin Hand Function Scale (19.24 vs 12.48, ES: 0.48), Scleroderma Health Assessment Questionnaire (0.95 vs 0.48, ES: 1.01), delta finger-to-palm (92.86 vs 106.33, ES: 0.40), grip strength (14.43 vs 19, ES: 0.58), tip pinch strength (2.49 vs 4.18, ES: 1.15), key pinch strength (4.01 vs 5.22, ES: 0.76), Raynaud phenomenon impact (0.94 vs 0.47, ES: 0.75), Short Form Health Survey-role physical (47.38 vs 60.14, ES: 0.61), physical functioning (34.62 vs 61.9, ES: 0.18), social functioning (60.71 vs 75.6, ES: 0.64), bodily pain (50.55 vs 63.38, ES: 0.58), vitality (45.95 vs 62, ES: 2.22), mental health (56.62 vs 72.38, ES: 0.84) moisturizing, and cold avoidance habits. Patients considered the program easy to follow with no adverse effects related to exercises. DISCUSSION: We developed a home based hand care program to be offered to SSc patients. Improvements in hand function, strength, disability, motion, and overall quality of life were independent of age, income, education level, disease duration, and skin score. Our findings support those of other studies that reported the benefits of hand exercises in SSc. Some study limitations include the lack of a control group, the small number of subjects and the short-time follow up. CONCLUSIONS: This home-based program for patients with SSc improved hand pain, function, mobility, and strength at the end of 8 weeks. Patient adherence and sustained efficacy is still to be determined.

Effectiveness of Occupational Therapy Interventions for Adults With Systemic Lupus Erythematosus: A Systematic Review.

OBJECTIVE: Our objective was to assess the efficacy of occupational therapy-related interventions for adults with systemic lupus erythematosus (SLE). METHOD: We reviewed intervention studies published from 2000 to 2017. The method used for conducting the review was based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The PEDro scale was used to evaluate methodological quality. Risk of bias was appraised with methods described by the Cochrane Methods Group. RESULTS: The final analysis included 20 studies (10 physical activity and 10 psychoeducational). Moderate evidence supports physical activity to improve depression, fatigue, exercise tolerance, and function without exacerbation of disease symptoms. Strong evidence supports psychoeducational interventions using cognitive-behavioral approaches to improve pain, depression, anxiety, perceived stress, quality of life, and function. Moderate evidence supports patient education and self-management interventions for pain, depression, anxiety, perceived stress, quality of life, and function. CONCLUSION: Further research on occupation-based interventions for people with SLE is needed.

Occupational Therapy Interventions for Adults With Fibromyalgia.

Evidence Connection articles provide a clinical application of systematic reviews developed in conjunction with the American Occupational Therapy Association's (AOTA's) Evidence-Based Practice Project. In this Evidence Connection article, we describe a case report of a person recently diagnosed with fibromyalgia. The occupational therapy assessment and intervention process in the home setting is described. Findings from the systematic review (Poole & Siegel, 2017) on this topic were published in the January/February 2017 issue of the American Journal of Occupational Therapy and in AOTA's Occupational Therapy Practice Guidelines for Adults With Arthritis and Other Rheumatic Conditions (Poole et al., 2017). Each article in this series summarizes the evidence from the published reviews on a given topic and presents an application of the evidence to a related clinical case. Evidence Connection articles illustrate how the research evidence from the reviews can be used to inform and guide clinical reasoning.

"I'm still dad": The Impact of Scleroderma on being a Father.

The purpose of this study was to describe the experiences of fathers with scleroderma. Ten fathers with scleroderma were interviewed by telephone. Interviews were tape-recorded and transcribed verbatim. Two key themes emerged related to the emotional impact of the illness and the day to day realities of the illness with the unpredictability and rareness of the illness leading to ongoing feelings of isolation and fear of mortality. The negative influences of being a father with scleroderma included the inability of the fathers to participate in physical activities with their children such as outdoor sports and throwing balls. Being able to spend quality time with the child was a positive influence of the illness.

Effectiveness of Occupational Therapy Interventions for Adults With Fibromyalgia: A Systematic Review.

OBJECTIVE: This systematic review addresses the effectiveness of occupational therapy-related interventions for adults with fibromyalgia. METHOD: We examined the literature published between January 2000 and June 2014. A total of 322 abstracts from five databases were reviewed. Forty-two Level I studies met the inclusion criteria. Studies were evaluated primarily with regard to the following outcomes: daily activities, pain, depressive symptoms, fatigue, and sleep. RESULTS: Strong evidence was found for interventions categorized for this review as cognitive-behavioral interventions; relaxation and stress management; emotional disclosure; physical activity; and multidisciplinary interventions for improving daily living, pain, depressive symptoms, and fatigue. There was limited to no evidence for self-management, and few interventions resulted in better sleep. CONCLUSION: Although the evidence supports interventions within the scope of occupational therapy practice for people with fibromyalgia, few interventions were occupation based.

Effectiveness of Occupational Therapy Interventions for Adults With Rheumatoid Arthritis: A Systematic Review.

OBJECTIVE: We reviewed the efficacy of occupational therapy-related interventions for adults with rheumatoid arthritis. METHOD: We examined 51 Level I studies (19 physical activity, 32 psychoeducational) published 2000-2014 and identified from five databases. Interventions that focused solely on the upper or lower extremities were not included. RESULTS: Findings related to key outcomes (activities of daily living, ability, pain, fatigue, depression, self-efficacy, disease symptoms) are presented. Strong evidence supports the use of aerobic exercise, resistive exercise, and aquatic therapy. Mixed to limited evidence supports dynamic exercise, Tai Chi, and yoga. Among the psychoeducation interventions, strong evidence supports the use of patient education, self-management, cognitive-behavioral approaches, multidisciplinary approaches, and joint protection, and limited or mixed evidence supports the use of assistive technology and emotional disclosure. CONCLUSION: The evidence supports interventions within the scope of occupational therapy practice for rheumatoid arthritis, but few interventions were occupation based.

Activity Limitations, Participation, and Quality of Life in American Indians with and without Diabetes.

The objective of this study was to examine activity limitations, participation, and quality of life (QOL) in American Indians with and without diabetes. It was a cross-sectional study that included 43 participants with diabetes and 31 healthy controls. Participants received evaluations of QOL, activity and participation limitations and factors such as pain, joint motion, hand function, and depression. There were no significant differences between the two groups for past, present, or future global QOL. However, there were significant differences between the two groups for the activity and participation measures and all measures of body structure/function except for left hand strength and depression. Current health and pain, and activity limitation correlated with QOL in both groups. Joint motion and participation correlated with QOL in the group with diabetes. American Indians with diabetes had more impairments in body structure and function and limitations in activities and participation compared to American Indians without diabetes.

Reliability and validity of the Manual Ability Measure-36 in persons with Charcot-Marie-Tooth disease.

STUDY DESIGN: Descriptive, clinical measurement. INTRODUCTION: Charcot-Marie-Tooth disease (CMT) is a genetic disorder that results in demyelination or axonal degeneration in peripheral nerves. Characteristic symptoms include decreased muscle strength and sensation, particularly in the hands and feet. PURPOSE: Examine the reliability and validity of the Manual Ability Measure (MAM-36), a 36-item self-report questionnaire specific to hand function, in persons with CMT. METHODS: Fourteen participants with CMT completed the MAM-36 at two points one week apart. Participants were also administered performance-based tests for grip and pinch strength, dexterity, and sensation. RESULTS: The test-retest intraclass correlation coefficient (ICC) for the MAM-36 was 0.96. Pearson correlation coefficients showed fair to good relationships (r = 0.41-0.64) between the MAM-36 scores and most of the performance tests. CONCLUSION: The MAM-36 is a reliable and valid assessment tool for measuring hand function during everyday tasks in persons with CMT. LEVEL OF EVIDENCE: IIb.

Mothers with chronic disease: a comparison of parenting in mothers with systemic sclerosis and systemic lupus erythematosus.

This study compared parenting ability in mothers with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE), two chronic, autoimmune, connective tissue diseases that primarily affect women. Seventy-four mothers with SSc and 68 mothers with SLE completed self-report questionnaires on demographic characteristics, pain, fatigue, occupational performance, and parenting. Results showed that mothers with SSc were slightly older, were more educated, and had decreased occupational performance. Mothers with SLE had more pain and fatigue. There were no significant differences between mothers with SSc and SLE who had children 5 years of age and younger. However, there were significant differences between the mothers with SSc and SLE who had children aged 6 to 18 years on four items on the Parenting Disability Index: playing games with child, getting up with child during the night, keeping child out of unsafe situations, and helping child solve personal/social problems. These differences may be due to differences in the levels of pain, fatigue, and occupational performance, possibly stemming from differences in disease manifestations.

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.

Hand impairment and activity limitations in four chronic diseases.

STUDY DESIGN: Retrospective cohort. INTRODUCTION: Hand involvement in osteoarthritis (OA) and rheumatoid arthritis (RA) are well known to occupational and physical therapists; however, it is not known whether the impairments and activity limitations with diabetes (DMII) and systemic sclerosis (SSc) are as severe as those observed with OA and RA. PURPOSE: To compare the hand impairments and activity limitations in the 4 diseases. METHODS: A convenience sample of 156 participants received evaluations of hand impairments: strength, joint motion, and dexterity and completed a hand activity limitations questionnaire. RESULTS: The SSc and RA participants had weaker pinch, decreased joint motion and more activity limitations than the DMII and OA groups. There were no significant differences between the groups for right hand grip strength and pegboard dexterity, and applied dexterity. CONCLUSIONS: OA and DMII groups had significantly less impairments and activity limitations than the SSc and RA groups. LEVEL OF EVIDENCE: 2C.

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.

'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.

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.

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.