Functional Impairments and Rehabilitation Outcomes of Patients With Immunotherapy-Induced Acute Inflammatory Demyelinating Polyradiculoneuropathy, Myasthenia Gravis, and Myositis.

ABSTRACT: Immunotherapy has led to a higher survival rate among different oncological disease groups but also associated with adverse-related events in multiple organ systems. Immunotherapy-related musculoskeletal weakness often results in a loss of cancer survivors' physical function, ultimately impacting their independence and quality of life. This is a retrospective study of 24 cancer patients who were treated with immunotherapy either alone or in conjunction with other oncological treatments. Twelve subjects (50%) were found to have acute inflammatory demyelinating polyradiculopathy/Guillain-Barré syndrome, six (25%) myositis, two (8%) myasthenia gravis, two (8%) diagnosis of myositis/myasthenia gravis, and one (4%) Guillain-Barré syndrome/myasthenia gravis combination. Physical therapy was provided in 91.7% of the cases, and physiatrist was involved in 54% of the cases. Almost half (45%) were discharged home, six (25%) to acute inpatient rehabilitation, two (8%) to subacute rehabilitation, three (12.5%) to hospice, and two (8%) died. The average length of hospital stay was 30 days, and eight patients (33%) readmitted within 3 mos. Our findings highlight the severity of functional impairments and the need for early rehabilitation interventions.

Systematic review of physical exercise for patients with idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIM) are systemic autoimmune connective tissue diseases. The safety and effectiveness of exercise for patients with idiopathic inflammatory myopathies remains unclear. This study aimed to systematically review the evidence for physical exercise among patients with idiopathic inflammatory myopathies. Relevant experimental studies were identified through searching the PubMed, Cochrane, Embase, Scopus, and CINAHL databases, and studies involving any type of physical exercise for ≥1 month were considered. The primary outcome was muscle strength, and the secondary outcomes included aerobic fitness, functional performance, health status, quality of life, activities of daily living, pain, and fatigue. Eight randomized controlled trials and thirteen nonrandomized uncontrolled trials were reviewed. Physical exercise appeared safe, with several positive effects. However, selection or allocation biases and small sample sizes affected the certainty of the evidence. While physical exercise appeared safe for patients with idiopathic inflammatory myopathies with several positive effects, studies of a higher methodological quality and involving patients with active disease are needed. Furthermore, to design optimal exercise programs, consistent and sensitive outcome measures are needed to facilitate comparisons of results from different studies.

Exercise training attenuates insulin resistance and improves ß-cell function in patients with systemic autoimmune myopathies: a pilot study.

INTRODUCTION/OBJECTIVES: To assess the effects of exercise training on insulin resistance and ß-cell function in patients with systemic autoimmune myopathies (SAMs). METHOD: This quasi-experimental, prospective study includes 9 patients with SAMs (six with dermatomyositis, two with antisynthetase syndrome, and one with polymyositis). Patients were submitted to a 12-week, twice a week, exercise training program comprising aerobic and resistance exercises. Baseline and after the intervention, we evaluated disease status, aerobic capacity, muscle strength, body composition, insulin resistance, and ß-cell function parameters. RESULTS: The patients have a mean age of 46.7 years and stable disease. No clinical or laboratory parameter impairment was observed after the intervention. Compared with baseline, aerobic capacity, muscle strength, and function increased after 12 weeks (P < 0.05), while no changes were observed for body composition. Data from the oral glucose tolerance test showed that exercise did not change glucose area under the curve (AUC), whereas insulin and C-peptide AUC decreased significantly (P < 0.05). Furthermore, Matsuda index and HOMA2 percentage (both surrogates of insulin resistance) also improved (P < 0.05). CONCLUSION: Exercise training improved aerobic capacity, muscle strength, and muscle function in patients with SAMs. In addition, exercise training led to an attenuation of insulin resistance and improvements in ß-cell function parameters. These data indicate that exercise training can mitigate metabolic impairments, attenuating the cardiovascular risk in SAMs.Key Points• Exercise training improved aerobic capacity, muscle strength, and function without disease impairment• Exercise training was capable of improve insulin resistance and ß-cell function in patients with SAM• These results suggest that exercise can mitigate metabolic impairments in patients with SAM, attenuating the cardiovascular risk.

Physical therapy in adult inflammatory myopathy patients: a systematic review.

The safety and effect of physical therapy in adult patients with idiopathic inflammatory myopathies (IIMs) are currently unclear. Considering the muscle weakness resulting from disease activity as well as from the administered drugs, these patients could benefit from an evidence-based physical therapy program. To perform a systematic review to assess safety and effects of physical therapy on the functional outcome of patients with idiopathic inflammatory myopathies in both active and quiescent disease: Pubmed, Embase, and Cochrane. Patients with one of the following idiopathic inflammatory myopathies: polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, and/or overlap myositis. The intervention included several types of rehabilitation programs, from strength and resistance training to endurance training, with a minimal duration of 1 month. Studies reporting intervention-related adverse events, disease activity, and functional outcomes were eligible. The risk of bias was assessed using the Cochrane guidelines. We included five randomized controlled and seven open-label non-randomized non-controlled trials. Data on statistical significance were extracted for all the trials. Included trials were of medium-quality evidence given the low number of patients and some risk of bias factors. Physical therapy does not have a negative effect on the disease activity of idiopathic inflammatory myopathies in quiescent disease and could improve functional outcome. The physical therapy program should minimally include endurance training. A combination with resistance training might be beneficial.

Physical exercise among patients with systemic autoimmune myopathies.

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.

Revisión de las miopatías inflamatorias a propósito de 3 casos en unidades de recuperación funcional de hospitales de media estancia / Review of inflamatory myopathies based on 3 cases in functional recovery units in long-term care hospitals

Las miopatías inflamatorias idiopáticas son un grupo heterogéneo de enfermedades sistémicas de etiopatogenia autoinmune. Se caracterizan por debilidad muscular, alteraciones miopáticas en el electromiograma y elevación de las enzimas musculares. Pueden darse de forma aislada, o asociarse a otras enfermedades autoinmunes o a neoplasias. Realizamos una aproximación a estos procesos a propósito de 3 pacientes tratados en Unidades de Recuperación Funcional de 2 hospitales de media estancia. En todos se produce debilidad muscular bilateral y simétrica de predominio en cinturas, con grave repercusión funcional. Por este motivo precisan realizar tratamiento en régimen de ingreso hospitalario. El objetivo terapéutico es doble: por un lado, frenar de forma urgente la actividad autoinmune para evitar el daño muscular progresivo y, por el otro, instaurar un tratamiento rehabilitador de forma precoz que minimice el deterioro funcional. En todos los casos se obtiene beneficio de la intervención, ya que permite la vuelta al domicilio con distintos grados de independencia (AU)

Idiopathic inflammatory myopathies are a heterogeneous group of systemic autoimmune diseases characterised by bilateral and symmetric muscle weakness, myopathic alterations on electromyography and elevated muscle enzymes. They can be associated with other autoimmune diseases or neoplastic processes. We provide a review of these processes based on 3 patients in the Functional Recovery Units of 2 long-term care hospitals. All 3 patients had bilateral and symmetrical muscle weakness, predominantly in the waist, with severe functional impairment. Consequently, these patients required hospital admission. The goal of rehabilitation is twofold: on the one hand, to urgently halt the autoimmune activity in order to avoid further muscle damage, and, on the other hand, to start early rehabilitation treatment to minimise functional impairment. All 3 patients benefited from the intervention, allowing discharge to home, with varying degrees of independence (AU)

Postrehabilitation Functional Improvements in Patients With Inflammatory Myopathies: The Results of a Randomized Controlled Trial.

OBJECTIVE: To evaluate the medium-term functional effect and the effect on quality of life of a standardized rehabilitation program in patients with inflammatory myopathies (IMs). DESIGN: A multicenter, randomized controlled trial. SETTING: Four university hospitals. PARTICIPANTS: Patients (N=21) with polymyositis. INTERVENTIONS: The intervention group participated in a 4-week standardized, hospital-based rehabilitation program followed by a personalized, self-managed, home-based rehabilitation program. The control group received physiotherapy on an outpatient basis. Study participants were evaluated at inclusion, at the end of the rehabilitation program (1mo), and then at 6 and 12 months. MAIN OUTCOME MEASURES: The primary efficacy criterion was the Health Assessment Questionnaire Disability Index (HAQ-DI), and the secondary criteria were quality of life (according to the Medical Outcomes Study 36-Item Short-Form Health Survey [SF-36] questionnaire), muscle performance (isokinetic strength, Motor Function Measure, and Kendall Manual Muscle Test), gait, pain, fatigue, and biomarkers of tolerance and disease activity. RESULTS: At 12 months, the mean ± SD HAQ-DI was significantly lower in the intervention group than in the control group (.64±.53 vs 1.36±1.02; P=.026). The intervention group also had better scores than the control group for some quality-of-life dimensions (SF-36 General Health: 53.44±8.73 vs 36.57±22.10, respectively; P=.038; SF-36 Role Physical: 63.89±43.50 vs 17.86±37.40, respectively; P=.023) and pain levels (5.0±10.61 vs 33.38±35.68, respectively; P=.04) at 12 months. The program was well tolerated by all the participants. CONCLUSIONS: In patients with IMs, the combination of a 4-week standardized rehabilitation program and a personalized, home-based, self-managed rehabilitation program was well tolerated and had a positive medium-term functional effect.

[Usefulness of rehabilitation in inflammatory myopathies]. / Intérêt de la rééducation fonctionnelle au cours des myopathies inflammatoires.

Rehabilitation, for a long time not recommended, seems today to hold a prominent place within the therapeutic arsenal of inflammatory myopathies. The difficulty of its evaluation, apart from the low prevalence of these diseases, lies in a triple heterogeneity: first that distinguishing the different forms of inflammatory myopathies and the phase where they are active; second, that concerning the endpoint considered to assess the efficiency of the intervention; lastly, the diversity of the rehabilitation programs that can be undertaken. Between 1993 and 2016, about 30 studies estimating the rehabilitation of inflammatory myopathies have been published, among which five randomized controlled trials, four controlled trials, 15 open studies, and seven case reports. All these studies evidence the safety of rehabilitation and some show a significant improvement of the criteria estimating the activity of the disease, its functional impact or the impairment of quality of life and the limitation in daily life activities triggered by the disease. The rehabilitation, whether aerobic, anaerobic or mixed, must today systematically be associated with the pharmaceutic treatment proposed to patients affected by inflammatory myopathies. Other studies are necessary to optimize the rehabilitation methods, to understand their effects and action, and to quantify their impact and provide more trustworthy evidence.

Calcinosis en manos y síndrome antisintetasa sin afectación muscular / Calcinosis in hands and antisynthetase syndrome without muscle involvement

No disponible

Proposal for a Candidate Core Set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies.

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 with IIM.

New insights into the benefits of exercise for muscle health in patients with idiopathic inflammatory myositis.

With recommended treatment, a majority with idiopathic inflammatory myopathy (IIM) develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis and to some extent in active disease and inclusion body myositis (IBM). Importantly, randomized controlled trials (RCTs) indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking mechanisms underlying effects of exercise in skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes in aerobic phenotype and muscle growth programs, and down regulation in genes related to inflammation. Altogether, exercise contributes to both systemic and within-muscle adaptations demonstrating that exercise is fundamental to improve muscle performance and health in IIM. There is a need for RCTs to study effects of exercise in active disease and IBM.

Exercise in neuromuscular diseases.

This article reviews the current knowledge regarding the benefits and contraindications of exercise on individuals with neuromuscular diseases (NMDs). Specific exercise prescriptions for individuals with NMDs do not exist because the evidence base is limited. Understanding the effect of exercise on individuals with NMDs requires the implementation of a series of multicenter, randomized controlled trials that are sufficiently powered and use reliable and valid outcome measures to assess the effect of exercise interventions-a major effort for each NMD. In addition to traditional measures of exercise efficacy, outcome variables should include measures of functional status and health-related quality of life.

Exercise in inflammatory myopathies, including inclusion body myositis.

Exercise is an important part of treatment in patients with idiopathic inflammatory myopathies. Improved functioning, ability to perform activities of daily living, and health-related quality of life have been reported in adult polymyositis, dermatomyositis, and also recently inclusion body myositis following different exercise regimens, with no signs of increased muscle inflammation. Intensive resistance training could reduce clinical disease activity and reduce expression of genes regulating inflammation and fibrosis in chronic polymyositis and dermatomyositis. Today, exercise research in adult myositis is focused on understanding mechanisms for muscle impairment and improved muscle function in relation to exercise and verifying results from small, open studies in larger settings. There are no studies evaluating the effects of exercise over weeks or months in juvenile dermatomyositis, other than a case report; however, there is to our knowledge an ongoing effort to evaluate the safety and effects of exercise in patients with juvenile dermatomyositis.

[Exercises in patients with myositis--active treatment intervention?]. / Vjezbe kod miozitisa--aktivna terapijska mjera?

Polymyositis, dermatomyositis and inclusion body myositis are rare inflammatory myopathies characterized by muscle weakness. Regardless of pharmacological treatment in most patients remain muscle weakness and inability to perform daily activities. Until recently, the prevailing opinion was that active exercises can exacerbate the inflammatory activity in the muscles and is now known that active exercise and exercise with resistance improve strength and endurance of muscles, aerobic capacity and overall functional ability. Exercises are prescribed according to the disease activity, manual muscle test or dynamometer measurements, range of motion, cardiorespiratory capacity and clinical status of the locomotor system. Each of the components can be influenced by targeted exercises and should be a integral part of myositis therapy.

Safety and efficacy of exercise training in patients with an idiopathic inflammatory myopathy--a systematic review.

OBJECTIVE: Idiopathic inflammatory myopathies (IIMs) are a group of rare heterogeneous autoimmune skeletal muscle disorders characterized by muscle weakness, excessive muscle fatigue and diminished aerobic fitness. Exercise training could be one way to prevent or delay the negative effects of the disease and the impairments seen in patients with an IIM. The objective was to examine whether exercise training is safe and effective in patients with an IIM. METHODS: All experimental studies that assessed the safety and/or efficacy of an exercise training programme in patients with an IIM except for case studies were reviewed. Pre-MEDLINE, MEDLINE and EMBASE database searching was done up to November 2010. Information was extracted on the number of participants, characteristics of participants, type of intervention, type of outcome measure, type of study design, report characteristics, geographical origin and risk of bias within studies. The change (percentage and significance) in group mean or median for each outcome measure in each study was determined as well. RESULTS: Two randomized controlled trials, one non-randomized controlled trial and nine uncontrolled trials were included. No studies in children were found. Safety measures did not worsen and efficacy measures improved or did not change. Most of the included studies had a high selection and/or allocation bias. CONCLUSIONS: In conclusion, it appears that exercise training is safe and effective in adult patients with active as well as inactive stable IIMs. However, more studies with a well-controlled design are needed. In addition, studies in children with an IIM are indicated.

Medición manual de potenciales de unidad motora «miopáticos» / Manual measurements of potentials of the «myopathic» motor units

Objetivo. Investigar, en pacientes con miopatía, la posible existencia de potenciales de unidad motora (PUM) cuya duración individual esté por debajo de un límite normal y, por lo tanto, con un significado patológico y posiblemente miopático. Material y método. Se midió en tibialis anterior la duración individual de 20 PUM en 82 sujetos sanos, tomando en cada sujeto el PUM con la menor duración. Lo mismo se llevó a cabo en 24 pacientes diagnosticados de miopatía, en su caso tomando todos los PUM con duración indvidual por debajo del valor mínimo encontrado en sujetos sanos. Resultados. Duración individual mínima encontrada en sujetos sanos: 6,8ms. Número de PUM de 6,7ms o menos encontrados en tibialis anterior de sujetos con miopatía: 17 (el 33% de los sujetos con miopatía). Se compararon las duraciones mínimas entre sujetos sanos y enfermos, revelándose una diferencia significativa entre ambas, que se consideró patológica y específicamente miopática en el caso de los sujetos con miopatía. Conclusiones. Dado el significado probablemente patológico y específicamente miopático de los PUM por debajo de 6,8ms encontrados en tibialis anterior de los sujetos con miopatía, se propone el término «PUM miopático» para este tipo de PUM, así como el uso de este hallazgo como un criterio electromiográfico específico en el diagnóstico de las miopatías (AU)

Objective. To investigate in patients with myopathy the possible existence of motor unit action potentials (MUAPs) whose individual duration is below a normal limit, and therefore patients having a pathological and possibly myopathic significance. Material and method. The individual duration of 20 MUAPs was measured in the tibialis anterior of 82 healthy subjects, using the MUAP with the shortest duration from each subject. The same measurement was obtained for 24 patients diagnosed of myopathy, in these cases, using the MUAPs with individual duration below the minimum value found in the healthy subjects. Results. The minimum individual duration found in healthy subjects was 6.8ms. Number of MUAPs of 6.7ms or less found in tibialis anterior of subjects with myopathy: 17 (33% of the subjects with myopathy). The minimum durations were compared between both healthy and ill subjects, there being significant differences between both, considered as pathological and specifically myopathic in the case of the subjects with myopathy. Conclusions. Given the probably pathological and specifically myopathic significance of the MUAPs below 6.8ms found in the tibialis anterior of the subjects with myopathy, the term «myopathic» MUAP is proposed herein for this type of MUAPs. The use of this finding as a specific electromyographic criterion for the diagnosis of myopathies is also proposed (AU)

Haematological effect of pulsed ultrasound in acute muscular inflammation in rats.

OBJECTIVE: To evaluate the effect of low-intensity pulsed ultrasound (LIPUS) on the haematological dynamics of an acute inflammatory process after an iatrogenic muscular lesion. DESIGN: Controlled laboratory study. SETTING: Research laboratory. PARTICIPANTS: Eighteen male Wistar rats (weight 350 to 450g) were submitted to surgical incision in the biceps femoris muscle (approximately 50%), and subsequently subdivided into control (n=9) and LIPUS (n=9) groups. INTERVENTION: Ultrasound (1.0MHz) was applied at the lesion site in the pulsed mode (2ms on, 8ms off) at 0.4W/cm(2) for 3 minutes at 1, 8 and 24 hours post-surgery. MAIN OUTCOME MEASURES: In these periods, blood was collected through venepuncture of the retro-orbital plexus and evaluated for different types of leukocytes and erythrocytes. RESULTS: LIPUS reduced the total leukocyte count at 1, 8 and 24 hours post-surgery (1 hour, control 9017.2±481×10(3)/mm(3) vs LIPUS 6189.8±450×10(3)/mm(3); 8 hours, control 8078.2±501×10(3)/mm(3) vs LIPUS 5371.3±378×10(3)/mm(3); 24 hours, control 8192.3±646×10(3)/mm(3) vs LIPUS 6059.1±503×10(3)/mm(3); P<0.001). The monocyte count was reduced at 8 and 24 hours post-surgery (8 hours, control 815.5±126×10(3)/mm(3) vs LIPUS 375.4±70×10(3)/mm(3); 24 hours, control 875.3±124×10(3)/mm(3) vs LIPUS 564.7±56×10(3)/mm(3); P<0.001). The number of segmented neutrophils was only reduced at 1 hour post-surgery (control 5033.1±397×10(3)/mm(3) vs LIPUS 3594.8±191×10(3)/mm(3); P=0.006), and the lymphocyte count was only reduced at 8 hours post-surgery (control 4759.7±459×10(3)/mm(3) vs LIPUS 2584.1±356×10(3)/mm(3); P=0.003). Changes were not observed in the concentrations of young neutrophils, polibocytes and erythrocytes. CONCLUSION: LIPUS reduced aspects of the inflammatory process following an acute incisional muscular lesion.

Tratamiento rehabilitador en la miositis aguda benigna infantil / Rehabilitation treatment in benign acute childhood myositis

La miositis aguda benigna infantil es un proceso poco frecuente, transitorio y autolimitado que afecta a niños en edad escolar (predominantemente chicos) tras una infección respiratoria de vías altas. Se caracteriza por el inicio súbito de dolor intenso en las extremidades inferiores y dificultad o imposibilidad para caminar con elevación sérica de niveles de creatinquinasa. Presentamos el caso de una niña de 8 años afecta de miositis aguda benigna infantil. Describimos el cuadro clínico, las claves para el diagnóstico diferencial con otros procesos más graves que asocian incapacidad para caminar y evaluamos los beneficios del tratamiento rehabilitador en los casos atípicos o complicados para facilitar la resolución del proceso y evitar las complicaciones(AU)

Benign acute childhood myositis (BACM) is a rare, temporary and self-limited condition, affecting school-age children (generally boys) after upper respiratory infection. It is characterized by sudden onset of severe pain in the legs and difficulty or inability to walk, with increase in serum creatine kinase (CK) levels. We present the case of an 8-year-old girl with BACM and describe the clinical picture, keys for the differential diagnosis with other more serious disorders that associate inability to walk. We evaluate the benefits of rehabilitation treatment in the atypical or complicated cases in order to provide the solution to the condition and avoid complications(AU)