Patient Preference Assessment Reveals Disease Aspects Not Covered by Recommended Outcomes in Polymyositis and Dermatomyositis

Objectives. Polymyositis (PM) and dermatomyositis (DM) are characterized by impaired muscle function with a majority of patients developing sustained disability. The aim of this study was to evaluate the patient's individual priorities (patient preference) of disabilities most important to improve in PM/DM using the MacMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR), to correlate the MACTAR to myositis outcomes and to evaluate its test-retest reliability. Methods. Twenty-eight patients with PM/DM performed recommended outcomes as well as the MACTAR, which was performed twice with one week apart. Results. Sexual activity, walking, biking, social activities, and sleep constituted the predominating disabilities. Seventy-two and 33% of the identified disabilities were not covered by items of the Health Assessment Questionnaire and the Myositis Activities Profile. Correlations between the MACTAR and health-related quality of life measures were rs = −0.67–0.73, correlations with measures of activities of daily living and participation in society were rs = 0.51–0.60 with lower correlations for other outcomes. Intraclass correlation (ICC) and weighted Kappa (Kw) coefficients were 0.83 and 0.68, respectively, for test-retest reliability of the MACTAR. Conclusions. The MACTAR interview had promising measurement properties and identified patient preference disabilities in PM/DM that were not covered by recommended outcomes

Effects on muscle tissue remodeling and lipid metabolism in muscle tissue from adult patients with polymyositis or dermatomyositis treated with immunosuppressive agents.

BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are autoimmune muscle diseases, conventionally treated with high doses of glucocorticoids in combination with immunosuppressive drugs. Treatment is often dissatisfying, with persisting muscle impairment. We aimed to investigate molecular mechanisms that might contribute to the persisting muscle impairment despite immunosuppressive treatment in adult patients with PM or DM using gene expression profiling of repeated muscle biopsies. METHODS: Paired skeletal muscle biopsies from six newly diagnosed adult patients with DM or PM taken before and after conventional immunosuppressive treatment were examined by gene expression microarray analysis. Selected genes that displayed changes in expression were analyzed by Western blot. Muscle biopsy sections were evaluated for inflammation, T lymphocytes (CD3), macrophages (CD68), major histocompatibility complex (MHC) class I expression and fiber type composition. RESULTS: After treatment, genes related to immune response and inflammation, including inflammasome pathways and interferon, were downregulated. This was confirmed at the protein level for AIM-2 and caspase-1 in the inflammasome pathway. Changes in genes involved in muscle tissue remodeling suggested a negative effect on muscle regeneration and growth. Gene markers for fast type II fibers were upregulated and fiber composition was switched towards type II fibers in response to treatment. The expression of genes involved in lipid metabolism was altered, suggesting a potential lipotoxic effect on muscles of the immunosuppressive treatment. CONCLUSION: The anti-inflammatory effect of immunosuppressive treatment was combined with negative effects on genes involved in muscle tissue remodeling and lipid metabolism, suggesting a negative effect on recovery of muscle performance which may contribute to persisting muscle impairment in adult patients with DM and PM

Personal non-commercial use only

ABSTRACT. Objective. Currently there are no evidence-based recommendations regarding fitness and strength tests for patients with childhood or adult idiopathic inflammatory myopathies (IIM). This hinders clinicians and researchers in choosing the appropriate fitness-or muscle strength-related outcome measures for these patients. Through a Delphi survey, we aimed to identify a candidate core set of fitness and strength tests for children and adults with IIM. Methods. Fifteen experts participated in a Delphi survey that consisted of 5 stages to achieve a consensus. Using an extensive search of published literature and through the work of experts, a candidate core set based on expert opinion and clinimetrics properties was developed. Members of the International Myositis Assessment and Clinical Studies Group were invited to review this candidate core set during the final stage, which led to a final candidate core set. Results. A core set of fitness-and strength-related outcome measures was identified for children and adults with IIM. For both children and adults, different tests were identified and selected for maximal aerobic fitness, submaximal aerobic fitness, anaerobic fitness, muscle strength tests, and muscle function tests. Conclusion. The core set of fitness-and strength-related outcome measures provided by this expert consensus process will assist practitioners and researchers in deciding which tests to use in patients with IIM. This will improve the uniformity of fitness and strength tests across studies, thereby facilitating the comparison of study results and therapeutic exercise program outcomes among patients wit

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

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 AD , 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

Impact of COVID-19 on cardiovascular testing in the United States versus the rest of the world

Objectives: This study sought to quantify and compare the decline in volumes of cardiovascular procedures between the United States and non-US institutions during the early phase of the coronavirus disease-2019 (COVID-19) pandemic. Background: The COVID-19 pandemic has disrupted the care of many non-COVID-19 illnesses. Reductions in diagnostic cardiovascular testing around the world have led to concerns over the implications of reduced testing for cardiovascular disease (CVD) morbidity and mortality. Methods: Data were submitted to the INCAPS-COVID (International Atomic Energy Agency Non-Invasive Cardiology Protocols Study of COVID-19), a multinational registry comprising 909 institutions in 108 countries (including 155 facilities in 40 U.S. states), assessing the impact of the COVID-19 pandemic on volumes of diagnostic cardiovascular procedures. Data were obtained for April 2020 and compared with volumes of baseline procedures from March 2019. We compared laboratory characteristics, practices, and procedure volumes between U.S. and non-U.S. facilities and between U.S. geographic regions and identified factors associated with volume reduction in the United States. Results: Reductions in the volumes of procedures in the United States were similar to those in non-U.S. facilities (68% vs. 63%, respectively; p = 0.237), although U.S. facilities reported greater reductions in invasive coronary angiography (69% vs. 53%, respectively; p < 0.001). Significantly more U.S. facilities reported increased use of telehealth and patient screening measures than non-U.S. facilities, such as temperature checks, symptom screenings, and COVID-19 testing. Reductions in volumes of procedures differed between U.S. regions, with larger declines observed in the Northeast (76%) and Midwest (74%) than in the South (62%) and West (44%). Prevalence of COVID-19, staff redeployments, outpatient centers, and urban centers were associated with greater reductions in volume in U.S. facilities in a multivariable analysis. Conclusions: We observed marked reductions in U.S. cardiovascular testing in the early phase of the pandemic and significant variability between U.S. regions. The association between reductions of volumes and COVID-19 prevalence in the United States highlighted the need for proactive efforts to maintain access to cardiovascular testing in areas most affected by outbreaks of COVID-19 infection

Exercise and outcome measures in patients with polymyositis and dermatomyositis

Polymyositis (PM) and dermatomyositis (DM) are chronic idiopathic inflammatory myopathies (IIM) which are clinically characterized by symmetrical proximal muscle weakness, fatigue, myalgia and extra- muscular involvement. Muscle impairment is the most significant feature of PM and DM with characteristic infiltrates of mononuclear inflammatory cells in muscle biopsies, elevated inflammatory parameters in serum and characteristic EMG changes, which separate these disorders from neurologic disorders and also fibromyalgia. First-line pharmaceutical treatment is oral corticosteroids together with other immunosuppressive agents. Despite an initial favorable response to treatment most patients develop sustained disability. Historically, these patients have been discouraged from active exercise due to a fear of exacerbated disease activity and little is known of the potential effects of exercise. The lack of valid and reliable outcome measures for patients with PM and DM for assessment of impairment, activity limitation and participation restriction limit the ability to evaluate different types of interventions in these patients. The aim of this thesis was to develop and evaluate exercise regimens regarding both safety and efficacy for patients with chronic as well as active, recent onset PM and DM. Another objective was to develop disease-specific outcome measures and to evaluate their measurement properties. An easy to moderate home exercise program was performed by patients with both chronic and active PM or DM five days a week for 12 weeks. An intensive resistive exercise program with a load of 10 voluntary repetition maximum (VRM) in five muscle groups was also performed by patients with chronic PM or DM three days a week for seven weeks. Assessments of disease activity and disability were conducted. A disease-specific, self-administrated questionnaire to assess activity limitation, the Myositis Activities Profile (MAP) was developed based on activities presented in the ICIDH-2 beta-2 draft. The Functional Index 2 (FI-2) was developed based on the original FI to assess impairment. These two outcome measures were evaluated for different aspects of validity and reliability. The home exercise program could be safely employed in patients with both chronic and active disease, as no signs of increased muscle inflammation could be detected either by analyses of muscle biopsies, Magnetic resonance imaging (MRI) or CPK-levels. The patient groups improved with significantly reduced disability. The intensive resistive 7-week exercise program resulted in significantly reduced muscular impairment without any signs of increased disease activity as assessed by muscle biopsies and CPK-levels. Both the MAP and the FI-2 had satisfactory content validity, construct validity and reliability. In summary, patients with chronic as well as active, recent onset PM or DM can perform individualized active exercise without increased muscle inflammation and with positive effects on muscle impairment, activity limitation and participation restriction. Active exercise should be included in the rehabilitation as an addition to pharmacological treatment. More research needs to be conducted to further minimize the patients' persisting impairment and activity limitation. The MAP and the FI-2 are valid, reliable and feasible instruments for assessing activity limitation and impairment and are also sensitive to change in these patients

Validation of MAP Personal non-commercial use only. The Journal of Rheumatology United States

ABSTRACT. Objective. To evaluate some measurement properties of the Myositis Activities Profile (MAP) in adult patients with polymyositis (PM) and dermatomyositis (DM) in the United States. Methods. To assess content validity, patients with PM/DM rated difficulty and importance of items of the MAP using a visual analog scale (VAS), range 0-10. For construct validity, consecutive patients with PM/DM performed the 6-item core set for disease activity including the manual muscle test (MMT) and the Health Assessment Questionnaire (HAQ), the Functional Index-2 (FI-2; muscle endurance), and the MAP plus disease effect on well-being on a VAS. Item fit within subscales was analyzed by Cronbach&apos;s alpha. Patients with stable disease activity filled out the MAP again 1 week later

The McMaster Toronto Arthritis patient preference questionnaire (MACTAR): a methodological study of reliability and minimal detectable change after a 6 week-period of acupuncture treatment in patients with rheumatoid arthritis

Abstract Objectives The McMaster Toronto Arthritis patient preference questionnaire (MACTAR) is a semi-structured interview consisting of a baseline and a follow-up interview. The MACTAR baseline is reliable and valid, however the reliability of the MACTAR follow-up is scarcely described. The aim of this study was to describe aspects of reliability and ability to detect changes of the Swedish MACTAR follow-up following acupuncture treatment in individuals with rheumatoid arthritis. Results The study was of Single Subject Experimental Design, with a 2-week non-interventional A-phase and a 6-week intervention B-phase. Eight individuals with RA, age 30–68 years, were included. MACTAR baseline was performed once followed by five assessments with MACTAR follow-up during the A-phase and another ten assessments during the B-phase. Reliability statistics were calculated for measurements 1–3 during the A-phase and the ability to detect effects of acupuncture treatment was tested by celeration lines in the B-phase. The MACTAR follow-up was highly reliable (ICC = 0.7–0.9, SEM = 2.3–4.3, and SDD = 6.2–11.7). Visual and statistical analyses indicated that the MACTAR follow-up could detect effects on individual- and group levels after acupuncture treatment, indicating that the MACTAR follow-up seems to be reliable and is able to detect effects of acupuncture treatment in RA