Malin Regardt

Patients with polymyositis or dermatomyositis have reduced grip force and health-related quality of life in comparison with reference values: an observational study

OBJECTIVESnThe aims of this study were to investigate hand function in PM and DM patients and compare this with reference values in healthy individuals and also to investigate if hand function correlated with activity performance and health-related quality of life.nnnMETHODSnAn observational cross-sectional study was performed in 18 women and 13 men with PM or DM with established disease. Grip force and hand mobility were assessed by Grippit and Escola Paulista de Medicina-Range of Motion scale. Activity performance was measured with myositis activities profile and health-related quality of life by short form-36 (SF-36).nnnRESULTSnWomen and men with PM and DM with mean disease duration of 6.8 (5.5) years had a significantly lower grip force than gender- and age-matched healthy individuals (women 71% and men 60%). They also had significantly lower mean values in all dimensions of the health-related quality of life instrument SF-36 compared with the Swedish population. In patients with PM and DM, the grip force correlated significantly with the ability to perform domestic activities. In women with PM and DM, the grip force correlated significantly with the health-related quality of life dimensions vitality and mental health. There were no significant differences between patients with PM and DM regarding grip force, hand mobility, activity performance or health-related quality of life.nnnCONCLUSIONSnPatients with PM or DM have reduced grip force that could influence activity performance and health-related quality of life.

Improvement in Health and Possible Reduction in Disease Activity Using Endurance Exercise in Patients With Established Polymyositis and Dermatomyositis : A Multicenter Randomized Controlled Trial With a 1-Year Open Extension Followup

To determine the effects of a 12‐week endurance exercise program on health, disability, VO2 max, and disease activity in a multicenter randomized controlled trial in patients with established polymyositis (PM) and dermatomyositis (DM), and to evaluate health and disability in a 1‐year open extension study.

Patients’ Experience of Myositis and Further Validation of a Myositis-specific Patient Reported Outcome Measure — Establishing Core Domains and Expanding Patient Input on Clinical Assessment in Myositis. Report from OMERACT 12

Objective. The Outcome Measures in Rheumatology (OMERACT) myositis working group was established to examine patient-reported outcomes (PRO) as well as to validate patient-reported outcome measures (PROM) in myositis. Methods. Qualitative studies using focus group interviews and cognitive debriefing of the myositis-specific Myositis Activities Profile (MAP) were used to explore the experience of adults living with polymyositis (PM) and dermatomyositis (DM). Results. Preliminary results underscore the importance of patient input in the development of PROM to ensure content validity. Results from multicenter focus groups indicate the range of symptoms experienced including pain, fatigue, and impaired cognitive function, which are not currently assessed in myositis. Preliminary cognitive debriefing of the MAP indicated that while content was deemed relevant and important, several activities were not included; and that questionnaire construction and wording may benefit from revision. A research agenda was developed to continue work toward optimizing PRO assessment in myositis with 2 work streams. The first would continue to conduct and analyze focus groups until saturation in the thematic analysis was achieved to develop a framework that encompassed the patient-relevant aspects of myositis. The second would continue cognitive debriefing of the MAP to identify potential areas for revision. There was agreement that further work would be needed for inclusion body myositis and juvenile dermatomyositis, and that the inclusion of additional contributors such as caregivers and individuals from the pharmaceutical/regulatory spheres would be desirable. Conclusions. The currently used PROM do not assess symptoms or the effects of disease that are most important to patients; this emphasizes the necessity of patient involvement. Our work provides concrete examples for PRO identification.

Hand Exercise Intervention in Patients with Polymyositis and Dermatomyositis: A Pilot Study

OBJECTIVEnThe aim of the present study was to develop a 12-week hand exercise intervention for patients with polymyositis (PM) and dermatomyositis (DM) and evaluate adherence, patients opinions of the programme design and overall feasibility, and the effect on hand function and activity limitation after the intervention.nnnMETHODnA pilot hand exercise intervention was conducted on a convenience sample of 15 patients with reduced handgrip strength and established, inactive PM and DM. Acceptable adherence was set at 75%. The programme was evaluated based on patients opinions regarding exertion, the movements involved and overall feasibility. Hand- and pinch-grip strength, grip ability, dexterity and activity limitation were assessed.nnnRESULTSnEleven of 15 patients completed the intervention, with acceptable adherence of 78-100%. Measures of handgrip strength, dexterity and activity limitation were reduced at baseline compared with normative data from the literature. Throughout the intervention, rates of perceived exertion were scored between moderate and fairly strong. Finger abduction and adduction were excluded from the hand exercise programme because they were not feasible to perform. Repetitions of the exercise increased gradually to a maximum of 30 per movement. Patients regarded this as too time-consuming and suggested ten repetitions daily or 10-20 repetitions 2-4 times per week. There were some individual, clinically meaningful improvements in hand function and activity limitation. A comparison between baseline and after the intervention showed that the three-jaw (tripod) pinch-grip strength (left hand) had increased (pu2009<u20090.007; zu2009=u2009-2.7).nnnCONCLUSIONnA hand exercise programme was found to be feasible to perform by patients with established PM or DM. The effect was limited, with few individual improvements in hand function and activity limitation, indicating a need to increase the resistance in the movements and to limit the duration of each exercise session.

Muscle Strength and Muscle Endurance During the First Year of Treatment of Polymyositis and Dermatomyositis: A Prospective Study

Objective. To investigate muscle impairment (isometric and dynamic) and disease activity during the first year after diagnosis of polymyositis (PM) and dermatomyositis (DM), and to study the relationship between muscle impairment, patient-reported health, and disease activity. Methods. Seventy-two patients enrolled in the Swedish Myositis Register, 2003–2010, were followed prospectively. The Manual Muscle test (MMT-8; isometric muscle strength), the Functional Index of myositis test (FI-2; dynamic, repetitive muscle function), and disease activity (6-item core set) were retrieved at the time of diagnosis, and after 6 and 12 months. Self-reported health (Medical Outcomes Study Short Form-36; SF-36) was retrieved at 12 months. Results. At the time of diagnosis, median (Q1–Q3) for the FI-2 was 27.2% (7.9–60.5%) of maximal score compared to 93.8% (92.5–98.8%) of maximal MMT-8. At 12 months, the FI-2 and the MMT-8 improved to 29.4% (16.5–60.7%; p < 0.05) and 96.1% (88.1–99.4%), respectively (p < 0.01). At 12 months, 45% of patients improved ≥ 20%, and 27% worsened ≥ 20% in FI-2 score, while 10% improved ≥ 20% in MMT-8. Physician’s global visual analog scale (VAS), Health Assessment Questionnaire, and creatine phosphokinase levels improved significantly at 12 months (p < 0.05–0.001) while patient’s global and extramuscular VAS remained unchanged. The SF-36 physical function correlated strongly with the FI-2 (rs = 0.74; CI 0.55–0.85) and moderately with the MMT (rs = 0.54; CI 0.27–0.73), with lower correlations between muscle function and other SF-36 domains. Conclusion. Patients with PM/DM were characterized by impaired dynamic repetitive muscle function (DRMF) that correlated well with patient-reported physical function. Assessment of DRMF adds information regarding muscle impairment in these patients.

Advancing the Development of Patient-reported Outcomes for Adult Myositis at OMERACT 2016: An International Delphi Study

Objective. To define a set of core patient-reported domains and respective instruments for use in idiopathic inflammatory myopathies (IIM). Previously, we reported a systematic literature review on patient-reported outcomes (PRO) in IIM followed by conducting international focus groups to elicit patient perspectives of myositis symptoms and effects. Methods. Based on qualitative content analysis of focus groups, an initial list of 26 candidate domains was constructed. We subsequently conducted an international modified Delphi survey to identify the importance of each of the 26 domains. Participants were asked to rate each domain on a scale of 0–10 (0 = not important, 10 = very important). Results. In this first round of the Delphi survey, 643 patients participated from the United States (n = 543), Sweden (n = 49), and South Korea (n = 51). Of the 26 domains, 19 (73%) were rated of high importance (≥ 7/10). The top 5 domains were muscle symptoms, fatigue, interactions with healthcare, medication side effects, and pain. During Outcome Measures in Rheumatology (OMERACT) 2016, we discussed the goal for ultimate reduction in the number of domains and the importance of considering representation of healthcare providers from other specialties, caregivers, representatives of pharmaceutical industries, and regulatory authorities in the next rounds of Delphi to represent broader perspectives on IIM. Conclusion. Further prioritization and a reduction in the number of domains will be needed for the next Delphi. At the next biennial OMERACT meeting, we aim to present and seek voting on a Myositis Preliminary PRO Core Set to enable ultimate measure selection and development.

Work ability in patients with polymyositis and dermatomyositis: An explorative and descriptive study

BACKGROUNDnPolymyositis (PM) and dermatomyositis (DM) are rare, chronic inflammatory diseases leading to muscle weakness and low muscle endurance. The muscle weakness may lead to restrictions in daily activities and low health-related quality of life.nnnOBJECTIVESnThis study aimed to investigate the work situation, work ability, work-related risk factors, and influence of the physical and psycho-social work environment in patients with PM and DM.nnnMETHODSnPatients with PM/DM were assessed using the Work Ability Index (WAI), and the Work Environment Impact Scale (WEIS).nnnRESULTSnForty-eight patients (PM n = 25 and DM n = 23) participated (women/men: 29/19) with a mean age of 54 years (range 28-67 years, SD.10) and mean disease duration of nine years (SD.9). Forty-four percent worked full-time, 31% part-time and 25% were on full-time sick leave. More than 50% self-rated work ability as poor or less good. Physically strenuous work components were present quite to very often in 23-79% and more in patients on sick leave ≥ 2 years. For those working, the interfering factors in the work environment concerned task and time demands. Supporting factors concerned meaning of work, interactions with co-workers and others. Self-rated work ability correlated moderately-highly positive with percentage of full-time employment, work-related risk factors and opportunities and constraints in the work environment.nnnCONCLUSIONSnPoor self-rated work ability is common in patients with PM/DM indicating a need to identify interfering risk factors and support patients to enhance work performance.

Perceptions of Patients, Caregivers, and Healthcare Providers of Idiopathic Inflammatory Myopathies: An International OMERACT Study

Objective. Patient-reported outcome measures (PROM) that incorporate the patient perspective have not been well established in idiopathic inflammatory myopathies (IIM). As part of our goal to develop IIM-specific PROM, the Outcome Measures in Rheumatology (OMERACT) Myositis special interest group sought to determine which aspects of disease and its effects are important to patients and healthcare providers (HCP). Methods. Based on a prior qualitative content analysis of focus groups, an initial list of 24 candidate domains was constructed. We subsequently conducted an international survey to identify the importance of each of the 24 domains to be assessed in clinical research. Patients with IIM, their caregivers, and HCP treating IIM completed the survey. Results. In this survey, a total of 638 respondents completed the survey, consisting of 510 patients, 101 HCP, and 27 caregivers from 48 countries. Overall, patients were more likely to rank “fatigue,” “cognitive impact,” and “difficulty sleeping” higher compared with HCP, who ranked “joint symptoms,” “lung symptoms,” and “dysphagia” higher. Both patients and providers rated muscle symptoms as their top domain. In general, patients from different countries were in agreement on which domains were most important. One notable exception was that patients from Sweden and the Netherlands ranked lung symptoms significantly higher compared to other countries including the United States and Australia (mean weighted rankings of 2.86 and 2.04 vs 0.76 and 0.80, respectively; p < 0.0001). Conclusion. Substantial differences exist in how IIM is perceived by patients compared to HCP, with different domains prioritized. In contrast, patients’ ratings across the world were largely similar.

AB1462-HPR Activity limitations and perceived health-related quality of life in people with inclusion body myositis – a cross-sectional study

Background Inclusion body myositis (IBM) is a slowly progressive inflammatory myopathy with muscle weakness around thighs, knees and finger flexion muscles. Most people are 50 years or older at onset and there are more men than women with ratio of 2–3: 1. The diagnosis leads to activity limitations in daily life. Objectives The aim of this study was to describe how individuals with IBM describe their activity limitation and how they estimate their perceived health-related quality of life as well as to describe the correlation between activity limitation and experienced health-related quality of life. Methods A quantitative cross-sectional study involving a total of 29 people with IBM participated. Data collection was performed in Stockholm Sweden. The Myositis Activities Profile (MAP) were used to measure activity limitation and the Short Form-36 (SF-36) were used to measure experienced health-related quality of life. Results Activity limitations were estimated from no difficulty to impossible to do. The activity with the highest value (very difficult) was movement. There was a significant difference between individuals with IBM and Swedish normative references measured with SF-36 in physical function, physical role function, general health and social function (p≤0.01). There were high correlations between SF-36 physical and social function with MAP’s movement, household activities, social activity, work/school and leisure (−0.872 ≥rs≤-0.702; p≤0.001). Conclusions Participants estimated activity limitations in many areas, of which movement was estimated as very difficult. The study demonstrated correlation between activity limitations and the experience of impaired health-related quality of life. In order to make positive impact on the health-related quality of life, it is important to focus on social areas such as family, friends and leisure activities, as well as optimising everyday living with proper mobility tools. Acknowledgements We would like to thank all the participant for taking time to be part of this study. We acknowledge The Myositis Association for making this study possible with a fellowship grant. Disclosure of Interest None declared

AB1248-HPR Work Ability in Patients with Polymyositis and Dermatomyositis: An Explorative and Descriptive Study

Background Polymyositis (PM) and dermatomyositis (DM) are characterized by muscle weakness which affects activity and participation. Work and leisure are the activities patients with PM/DM perceive as most limited. Previous studies addressing work in terms of how many are employed and degree of sick-leave. No study has investigated the self-rated work ability or how the work environment affects the ability to work in these patients. Objectives This study aimed to investigate the work situation, work ability, work-related risk factors, and influence of the physical and psycho-social work environment in patients with polymyositis (PM) and dermatomyositis (DM). Methods Patients with PM/DM were assessed using the Work Ability Index (WAI), and the Work Environment Impact Scale (WEIS). Results Forty-eight patients (PM n=25 and DM n=23) participated (women/men: 29/19) with a mean age of 54 years (range 28-67 years, SD.10) and mean disease duration of nine years (SD.9). Forty-four percent worked full-time, 31% part-time and 25% were on full-time sick leave. More than 50% self-rated work ability as “poor” or “less good”. Physically strenuous work components were present “quite to very often” in 23-79% and more in patients on sick leave ≥2 years. For those working, the interfering factors in the work environment concerned task and time demands. Supporting factors concerned meaning of work, interactions with co-workers and others. Self-rated work ability correlated moderately–highly positive with percentage of full-time employment, work-related risk factors and opportunities and constraints in the work environment. Conclusions Poor self-rated work ability is common in patients with PM/DM indicating a need to identify interfering risk factors and support patients to enhance work performance. Disclosure of Interest None declared