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PURPOSE OF REVIEW The aim of this review is to present the main pieces of evidence, recent literature and to present future perspectives on the use of exercise/physical training in the treatment and improvement of the quality of life of patients with systemic autoimmune myopathies. RECENT FINDINGS In the last decades, knowledge about the relevance of physical exercise training in preventing and treating chronic diseases and improving quality of life has grown. Following the global trend exemplified by the expression 'exercise is medicine', the importance of exercise/physical training has also grown in myopathies. However, the science of exercise has a lot to collaborate on and improve patients' quality of life with myopathies by appropriating new technological tools, including accessible and low-cost devices and smartphone apps. SUMMARY Physical exercise, as already consolidated in the literature, is an effective, well tolerated, and low-cost strategy for patients with myopathies. The use of wearable devices, smartphone apps, and online training prescriptions must accompany the global scenario, bringing new research fields and expanding the options for access to training for the individualized basis, and prescribed by qualified professionals.

Background: To evaluate aerobic capacity and muscle function in patients with Relapsing Polychondritis (RP). Methods: A cross-sectional single center study evaluated seven women with RP (McAdam criteria) that were compared to seven healthy control individuals matched for age, gender, and body mass index. Both groups performed a treadmill-graded maximal exercise. Aerobic capacity was assessed by peak oxygen uptake (VO2 peak), time at anaerobic Ventilatory Threshold (VAT) and Respiratory Compensation Point (RCP) and time-to-exhaustion. Muscle function was evaluated through the sit-to-stand and the timed up-and-go test.

Objectives Several studies have shown not only a high prevalence of fatigue but also a reduction in health-related quality of life (HRQoL) in patients with rheumatic diseases. Owing to insufficient research in this area, we aimed to assess the prevalence of fatigue and its contribution to impairment of HRQoL in patients with Takayasu arteritis (TAK). Methods This single-centre case–control study included 53 TAK patients who were matched by age, BMI and sex with 100 healthy individuals. Aside from the patients’ general data, the following information was collected: disease activity, level of activities of daily living (HAQ), physical activity levels and chronic fatigue. Results The TAK patients and healthy individuals were comparable in terms of current age, BMI and sex distribution. The median disease duration of TAK was 13.0 (7.0–20.0) years, and 11 (20.8%) patients had active disease. Compared with healthy individuals, patients with TAK had a higher prevalence of fatigue and lower HAQ score, physical activity level and intensity, and physical and psychosocial domains of the modified fatigue impact scale (P Conclusion TAK patients have a higher prevalence of fatigue, which affects different aspects of the disease, including physical function. Thus, fatigue-focused treatments should also be considered in clinical practice. Trial registration The Brazilian Clinical Trials Registry (ReBEC), https://ensaiosclinicos.gov.br/, RBR-9n4z2hh.

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Background/objective Patients with systemic autoimmune myopathies (SAMs) have high prevalence of dyslipidemia and, consequently, possible endothelial dysfunction and vascular stiffness. Our objective was to evaluate the possible benefits on endothelial function and vascular stiffness, as well as adverse effects of atorvastatin in SAMs. Methods A pilot longitudinal, double-blind, randomized, placebo-controlled study was conducted. Twenty-four of 242 patients were randomized at a 2:1 ratio to receive atorvastatin (20 mg/d) or placebo for a period of 12 weeks. Demographic data, comorbidities, and clinical and laboratory parameters, as well as endothelial function and arterial stiffness, were evaluated. Results Of the 24 randomized patients, 4 patients were excluded, with remaining 20 patients (14 in the atorvastatin group and 6 in the placebo group). The mean age of the patients was 49.0 years, and 75% of the patients were female. At baseline, the demographic data, disease status, treatment, cardiovascular comorbidities, and risk factors were comparable between the atorvastatin and placebo groups. After 12 weeks of follow-up of atorvastatin therapy, no improvements were observed for endothelial function and arterial stiffness in either group (p > 0.05). As expected, a significant reduction in total and low-density lipoprotein cholesterol levels was observed. During the study, no clinical intercurrences or disease relapses were observed in either group. Conclusions The atorvastatin drug attenuated low-density lipoprotein cholesterol without worsening clinical outcomes in SAMs. No change was observed for endothelial function and arterial stiffness. Additional studies, with long-term follow-up time and different atorvastatin dosage, are needed to corroborate the results of this study.

Transcranial direct current stimulation (tDCS) has emerged as a nonpharmacological tool in physical rehabilitation. There have currently no studies that evaluated the safety and efficacy of tDCS in patients with dermatomyositis. Case-report: Three adult women with dermatomyositis were allocated randomly to intervention (i-tDCS, one patient) or not (sham-tDCS, two patients) of three consecutive days of tDCS and evaluated in four periods: before-tDCS (PRE), 15 minutes after-tDCS (0th POST), 15 days after-tDCS (15th POST), and 30 days after-tDCS (30th POST). The tDCS was safe throughout the protocol, without disease relapsing or adverse effects related to tDCS. Furthermore, the tDCS increased the muscle torque and total work of dominant and non-dominant elbow flexors in the patient with i-tDCS, when compared to two patients with sham-tDCS. Conclusions: The tDCS was safe and appeared to influence long-term strength in the limb of the patient with stable dermatomyo-sitis.

BackgroundThis study was aimed at evaluating the aerobic capacity of patients with antisynthetase syndrome (ASS) and dermatomyositis (DM) and analyzing possible relationships between aerobic capacity and disease status, cardiovascular diseases and their risk factors.MethodsThe study was a cross-sectional, single-center study that assessed the aerobic capacity of 22 women (13 with DM and 9 with ASS) who were matched by age and body mass index to 17 healthy women (control group). The aerobic capacity (oxygen uptake [VO2peak], anaerobic threshold, respiratory compensation point and time-to-exhaustion) was evaluated using the cardiopulmonary treadmill test. Disease status was assessed using International Myositis Assessment & Clinical Studies Group (IMACS) set scores.ResultsThe patients had low IMACS parameters that showed low or absent disease activity. The distribution of cardiovascular diseases and their risk factors was similar between the patients and the control group (P > 0.05) at the time of the analysis. The patients with DM and the control group had similar aerobic capacity. However, the patients with ASS exhibited significantly reduced aerobic capacity (relative VO2peak, anaerobic threshold, respiratory compensation point and time to exhaustion) when compared to the control group. In addition, patients with ASS had a lower anaerobic threshold compared to the DM group. There were no significant relationships between the aerobic capacity and disease status, cardiovascular diseases and their risk factors.ConclusionIn contrast to DM patients and healthy individuals, patients with stable ASS have significantly impaired aerobic capacity, which is unlikely to be totally explained by traditional cardiovascular diseases, their risk factors and disease status. Further studies are needed to corroborate our data and to clarify the cause of this reduced aerobic capacity in ASS.

Systemic autoimmune myopathies (or idiopathic inflammatory myopathies) are a wide group of rare rheumatic diseases characterized by muscle weakness due to muscle inflammation. Recent evidence has continually reinforced the relevance of the perception of pain and fatigue as parameters of notable contribution to reducing the quality of life of these patients. However, due to the multidimensional characteristic of these variables, few studies address the effects of pharmacological and non-pharmacological therapies, such as exercise training on these variables. With regard to patients with systemic autoimmune myopathies, the outcomes related to the effects of exercise training are focused on muscle strength and endurance, few explore the effects on the perception of pain and fatigue. Given this, studies that review systematically the available evidence of the effects of exercise training on these perceptions can contribute to management of this parameter in clinical practice. Given this context, the present study aims to review systematically the effectiveness of exercise training specifically on the perception of pain and fatigue.

Background Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. Methods A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. Results Patients with TAK had a mean age of 41.5 (38.0–46.3) years, disease duration of 16.0 (9.5–20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P Conclusions TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.

Limb vascular claudication and hand muscle weakness are common symptoms of Takayasu arteritis (TAK). However, no studies have correlated these two symptoms. Therefore, the aim of the study was to evaluate handgrip strength and its correlation with both upper-limb vascular claudication and imaging of the vessels. This cross-sectional study compared 36 patients with TAK who were matched by age, gender, and body mass index with 36 individuals without TAK (CTR). Hand strength (assessed with handgrip dynamometer), functional capacity (Health Assessment Questionnaire, HAQ), upper-limb vascular claudication symptoms (patients’ selfreported form), and disease activity (Indian Takayasu Clinical Activity Score [ITAS] 2010; Physician Global Assessment [PGA], C-reactive protein, and erythrocyte sedimentation rate) were evaluated as well as vessel imaging (e.g., angiotomography or angioresonance) and blood pressure. The median age of the patients was 42.0 years (35.5-51.5 years), whereas the mean disease duration was 13.1±6.8 years. No patient had active disease. Compared to the CTR, the patients with TAK showed reduced strength in the left-hand (22.9±5.9 vs 26.3±5.6 kg; p=0.014) and increased HAQ scores [0.50 (0.12-0.87) vs 0.00 (0.00-0.00); p

Autoimmune rheumatic diseases (ARDs) have been closely associated with accelerated plaque progression and the development of atherosclerosis, which lead to high morbidity and mortality rates for cardiovascular diseases. Endothelial dysfunction and arterial stiffness are greatly evidenced in several studies in the early phase of atherosclerosis. In ARDs, endothelial dysfunction and arterial stiffness are related to traditional and non-traditional risk factors. To date, no studies have clearly analyzed the main parameter involved in endothelial dysfunction and arterial stiffness. In this context, the present narrative review’s purpose was to describe the main factor in endothelial dysfunction and arterial stiffness in different ARDs. Endothelial dysfunction and arterial stiffness are related to traditional risk factors (i.e., hypertension, diabetes, dyslipidemia, metabolic syndrome, sedentary behavior) and non-traditional risk factors (linked to the immune mechanisms involved in these diseases). Moreover, in the present study, these associations were systemically analyzed in ankylosing spondylitis, antiphospholipid syndrome, rheumatoid arthritis, psoriatic arthritis, systemic autoimmune myopathies, systemic lupus erythematosus and systemic sclerosis. The present review shows that the relationship of traditional risk factors and non-traditional risk factors related to ARDs works in the worsening of function and structural properties of arterial vessels, leading to high cardiovascular morbidity and mortality.

Introduction: Dermatomyositis (DM) and antisynthetase syndrome (ASS) show a high frequency of metabolic syndrome, which can be preceded by endothelial dysfunction and arterial stiffness. To date, only one study has evaluated these vessel parameters in DM, and no study of ASS exists. Therefore, the aim of the study was to assess the structural and functional arterial of arterial vessels in DM and ASS. Methods: This cross-sectional study enrolled 21 adult female patients (14 DM and 7 ASS) who were age-, gender- and ethnicity-matched to 12 healthy individuals. Patients using lipid lowering agents or prednisone at doses ≥ 0.25 mg/kg/day, and patients with uncontrolled systemic arterial hypertension, diabetes mellitus, cardiac insufficiency, and disease activity were excluded. Arterial stiffness was evaluated using carotid-femoral pulse wave velocity (PWV), and endothelial function was evaluated using dependent flow-mediated dilatation (FMD) of the brachial artery. Results: The mean age of patients with DM or ASS were 45.4 ± 8.6 and 44.0 ± 6.1 years, respectively (P = 1.000), and patients were predominantly of white ethnicity. Six DM patients and three ASS patients had systemic arterial hypertension, whereas 9 DM patients and six ASS patients had dyslipidemia. Endothelial baseline diameter, hyperemia diameter and FMD values were similar among the three groups (P > 0.05). Moreover, the median FMD values were also similar between the patients with DM and patients with ASS [8.3% (4.5% - 10.9%) vs. 6.0% (−1.8% - 8.2%); P = 0.585]. The PWV values were comparable among the three groups (P = 0.253). In addition, no difference was observed between patients with DM and patients with ASS (7.4 ± 0.8 m/s vs. 7.4 ± 0.9 m/s; P = 1.000). Conclusions: Despite the high prevalence of dyslipidemia and systemic arterial hypertension, our female patients with stable DM and ASS had FMD and PWV values comparable to those of the control group.

Introduction. Exercise training has been effective in improving endothelial function and decreasing arterial stiffness in several systemic autoimmune diseases. However, to date, no studies have assessed patients with systemic autoimmune myopathies (SAM). Methods. Five female patients with definite SAM (3 dermatomyositis and 2 polymyositis) who underwent a 12-week, twice-a-week, exercise training program were assessed prospectively. The following parameters were assessed: endothelial function measured by flow-mediated dilatation (FMD), carotid femoral by pulse wave velocity (PWV), disease status by International Myositis Assessment & Clinical Studies Group (IMACS) set score, the ventilatory anaerobic threefold (VAT), respiratory compensation point (RCP), maximum effort (ME), maximal oxygen uptake (VO2max) measured by ergospyrometer, and strength and muscle function. Results. Mean age of the patients and duration of disease were 43.9 and 10.5 years, respectively. The median FMD and PWV were 10.2% and 7.1 m/s. After exercise training, important FMD reduction was observed in 4 out of 5 patients, whereas the PWV parameters decreased in only 2 out of 5 patients. The exercise training was safe, without clinical intercurrences or disease relapses. Moreover, an increase in strength and functionality was observed. Concerning aerobic capacity, there was a decrease in the VAT and an increase in ME, without al-terations in the maximum oxygen consumption. Conclusions. In general, exercise training does not alter endothelial function and PWV values, but improves muscle strength and function and also, does not lead to disease reactivation (Clinical Trials (NCT03092167)).

Background:There is currently few information regarding rehabilitation in patients with systemic autoimmune myopathies (SAMs). Transcranial direct current stimulation (tDCS) has shown promising results for the motor performance of healthy individuals as for patients with, e.g., post-stroke hemiparetic limbs.Objectives:The present study was aimed to assessing the safety and efficiency of tDCS in patients with SAMs.Methods:This study is a prospective, randomized, sham controlled, double blind, and clinical trial with ethical approval. Eighteen adult patients with dermatomyositis, polymyositis, antisynthetase syndrome or immune-mediated necrotizing myopathies in remission or with minimal disease activity were enrolled from 2018 to 2019. Patients were allocated randomly in two groups to receive sham or active tDCS with 2mA amplitude submitted for 20 minutes for three consecutive days. The 5x7cm sponge-electrodes were positioned with the anode over the left (C1) or right (C2) - contralateral to the dominant limb, whereas the cathode over the FP2 or FP3, respectively (10-10 EEG electrodes placement). The groups were evaluated in four moments: pre-stimulation, and 30 minutes, 3 weeks and 8 weeks post-tDCS. They were evaluated in the different moments with International Myositis Assessment and Clinical Studies Group set scores, Short-Form health survey (SF-36), state-trait anxiety inventory (STAI), Beck depression inventory (BDI), timed up-and-go test (TUG), time-stands test (TST), isokinetic extension and flexion testing of bilateral knee and elbow. A specific security questionnaire for tDCS was used after the active or sham stimulation in all patients.Results:The demographic data, kind of myositis, disease duration, and disease status (all with low disease activity) were comparable between both active and sham tDCS groups. After interventions, there was improvement of values of patient’s VAS (P=0.042) and serum levels of creatine phosphokinase (P=0.005), independent of the group. Moreover, in active tDCS group, the physical aspects of SF-36 in week 8 (PPPPConclusion:This unprecedented study evidences the safety of tDCS, besides the potential efficiency in improving rehabilitation of tDCS in SAMs. More studies with a large samples and period of tDCS sessions are necessaries to corroborate with the present study.References:[1]Lundberg IE, et al. 2017 European league against rheumatism/ American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Arthritis Rheum. 2017;69:2271-82.[2]Tanaka S, et al. Single session of transcranial direct current stimulation transiently increases knee extensor force in patients with hemiparetic stroke. Neurorehabil Neural Repair. 2011;25:565-9.Acknowledgements:This work was supported by the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) 303.379/2018-9, Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) 2019/11776-6, Faculdade de Medicina da USP/SP to SKS.Disclosure of Interests:None declared

Systemic autoimmune myopathies (SAMs) are a heterogeneous group of rare systemic autoimmune diseases that primarily affect skeletal muscles. Patients with SAMs show progressive skeletal muscle weakness and consequent functional disabilities, low health quality, and sedentary lifestyles. In this context, exercise training emerges as a non-pharmacological therapy to improve muscle strength and function as well as the clinical aspects of these diseases. Because many have feared that physical exercise exacerbates inflammation and consequently worsens the clinical manifestations of SAMs, it is necessary to evaluate the possible benefits and safety of exercise training among these patients. The present study systematically reviews the evidence associated with physical training among patients with SAMs.

To evaluate the feasibility, safety and efficacy of exercise training in patients with immune-mediated necrotising myopathies (IMNM).Eight consecutive sedentary patients with IMNM (5 anti-signal recognition particle and 3 anti-hydroxy-methyl-glutaryl coenzyme A reductase) were engaged in this study. Disease status was based on International Myositis Assessment and Clinical Studies Group (IMACS) core set measures. Physical performance was evaluated by cardiopulmonary exercise test, repetition maximum (RM) protocol, handgrip dynamometry, sit-to-stand (STS) and timed up-and-go (TUG) tests. All these parameters were measured at baseline and after a 12-week, twice-a-week, supervised exercise training comprising aerobic and strength exercises.Patients (aged 61 years on average) were very disabled at the beginning of the disease (mean duration of 17.7 months), but after being aggressively treated with a treat-to-target approach, they presented only mild symptoms that were well-controlled with oral immunosuppression and low disease status scores by the time of the exercise intervention. No disease relapsing, worsening of the IMACS set scores or adverse events were observed throughout the training period. Patients also increased aerobic capacity (e.g. time to achieve anaerobic threshold and time to achieve exhaustion), muscle strength (e.g. 1RM bench press) and function (e.g. STS test).Supervised exercise training did not impair disease status and seemed to be feasible, safe and effective in patients with IMNM. Moreover, exercise training increased aerobic capacity, muscle strength and function, suggesting that this could be a novel potential coadjuvant therapy in IMNM.