Efficacy of aqua-based plyometric exercises in the rehabilitation of patients with juvenile dermatomyositis: A randomized crossover pilot study.

BACKGROUND: Patients with juvenile dermatomyositis (JDM) experience muscle weakness, tiredness, and loss of energy, which restrict their abilities in performance of their daily living activities. OBJECTIVE: To explore the effect of aquatic-based plyometric (Aqua-PLYO) exercises on muscle strength, fatigue, and functional ability in patients with JDM. METHODS: This was a randomized, single-blind, crossover pilot study that included 16 patients with JDM (age 13.44 ± 2.85 years). They were assigned randomly to receive either the Aqua-PLYO exercises (n = 8) or the standard outpatient care (SoC ; n = 8) first. After a 1-month washout, the treatment was reversed. Lower limb muscle strength, fatigue perception, functional ability, and disease activity were evaluated before and after each treatment period. RESULTS: Irrespective of the treatment order, the Aqua-PLYO treatment yielded greater improvement in muscle strength (hip flexors and abductors [P < 0.001] or knee flexors [P < 0.001] and extensors [P = 0.0008]), fatigue perception (P < 0.001), functional ability (P = 0.009), and disease activity (P = 0.0001) than the SoC treatment. By using the shortest confidence intervals (100[1-2α]%) of the difference, the average bioequivalence of the Aqua-PLYO and SoC has not been established at P = 0.05, because the upper and lower confidence bounds of all outcomes were not between the acceptance limits. No period or carryover effects were detected in all outcomes. CONCLUSION: The Aqua-PLYO exercise protocol as implemented in this study is safe, feasible, and well-tolerated in patients with JDM and seemingly useful to help increase muscle strength, reduce fatigue, and enhance functional ability in such a patient population.

Strength training and aerobic exercise training for muscle disease.

BACKGROUND: Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004 and last updated in 2013. We undertook an update to incorporate new evidence in this active area of research. OBJECTIVES: To assess the effects (benefits and harms) of strength training and aerobic exercise training in people with a muscle disease. SEARCH METHODS: We searched Cochrane Neuromuscular's Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL in November 2018 and clinical trials registries in December 2018. SELECTION CRITERIA: Randomised controlled trials (RCTs), quasi-RCTs or cross-over RCTs comparing strength or aerobic exercise training, or both lasting at least six weeks, to no training in people with a well-described muscle disease diagnosis. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included 14 trials of aerobic exercise, strength training, or both, with an exercise duration of eight to 52 weeks, which included 428 participants with facioscapulohumeral muscular dystrophy (FSHD), dermatomyositis, polymyositis, mitochondrial myopathy, Duchenne muscular dystrophy (DMD), or myotonic dystrophy. Risk of bias was variable, as blinding of participants was not possible, some trials did not blind outcome assessors, and some did not use an intention-to-treat analysis. Strength training compared to no training (3 trials) For participants with FSHD (35 participants), there was low-certainty evidence of little or no effect on dynamic strength of elbow flexors (MD 1.2 kgF, 95% CI -0.2 to 2.6), on isometric strength of elbow flexors (MD 0.5 kgF, 95% CI -0.7 to 1.8), and ankle dorsiflexors (MD 0.4 kgF, 95% CI -2.4 to 3.2), and on dynamic strength of ankle dorsiflexors (MD -0.4 kgF, 95% CI -2.3 to 1.4). For participants with myotonic dystrophy type 1 (35 participants), there was very low-certainty evidence of a slight improvement in isometric wrist extensor strength (MD 8.0 N, 95% CI 0.7 to 15.3) and of little or no effect on hand grip force (MD 6.0 N, 95% CI -6.7 to 18.7), pinch grip force (MD 1.0 N, 95% CI -3.3 to 5.3) and isometric wrist flexor force (MD 7.0 N, 95% CI -3.4 to 17.4). Aerobic exercise training compared to no training (5 trials) For participants with DMD there was very low-certainty evidence regarding the number of leg revolutions (MD 14.0, 95% CI -89.0 to 117.0; 23 participants) or arm revolutions (MD 34.8, 95% CI -68.2 to 137.8; 23 participants), during an assisted six-minute cycle test, and very low-certainty evidence regarding muscle strength (MD 1.7, 95% CI -1.9 to 5.3; 15 participants). For participants with FSHD, there was low-certainty evidence of improvement in aerobic capacity (MD 1.1 L/min, 95% CI 0.4 to 1.8, 38 participants) and of little or no effect on knee extension strength (MD 0.1 kg, 95% CI -0.7 to 0.9, 52 participants). For participants with dermatomyositis and polymyositis (14 participants), there was very low-certainty evidence regarding aerobic capacity (MD 14.6, 95% CI -1.0 to 30.2). Combined aerobic exercise and strength training compared to no training (6 trials) For participants with juvenile dermatomyositis (26 participants) there was low-certainty evidence of an improvement in knee extensor strength on the right (MD 36.0 N, 95% CI 25.0 to 47.1) and left (MD 17 N 95% CI 0.5 to 33.5), but low-certainty evidence of little or no effect on maximum force of hip flexors on the right (MD -9.0 N, 95% CI -22.4 to 4.4) or left (MD 6.0 N, 95% CI -6.6 to 18.6). This trial also provided low-certainty evidence of a slight decrease of aerobic capacity (MD -1.2 min, 95% CI -1.6 to 0.9). For participants with dermatomyositis and polymyositis (21 participants), we found very low-certainty evidence for slight increases in muscle strength as measured by dynamic strength of knee extensors on the right (MD 2.5 kg, 95% CI 1.8 to 3.3) and on the left (MD 2.7 kg, 95% CI 2.0 to 3.4) and no clear effect in isometric muscle strength of eight different muscles (MD 1.0, 95% CI -1.1 to 3.1). There was very low-certainty evidence that there may be an increase in aerobic capacity, as measured with time to exhaustion in an incremental cycle test (17.5 min, 95% CI 8.0 to 27.0) and power performed at VO2 max (maximal oxygen uptake) (18 W, 95% CI 15.0 to 21.0). For participants with mitochondrial myopathy (18 participants), we found very low-certainty evidence regarding shoulder muscle (MD -5.0 kg, 95% CI -14.7 to 4.7), pectoralis major muscle (MD 6.4 kg, 95% CI -2.9 to 15.7), and anterior arm muscle strength (MD 7.3 kg, 95% CI -2.9 to 17.5). We found very low-certainty evidence regarding aerobic capacity, as measured with mean time cycled (MD 23.7 min, 95% CI 2.6 to 44.8) and mean distance cycled until exhaustion (MD 9.7 km, 95% CI 1.5 to 17.9). One trial in myotonic dystrophy type 1 (35 participants) did not provide data on muscle strength or aerobic capacity following combined training. In this trial, muscle strength deteriorated in one person and one person had worse daytime sleepiness (very low-certainty evidence). For participants with FSHD (16 participants), we found very low-certainty evidence regarding muscle strength, aerobic capacity and VO2 peak; the results were very imprecise. Most trials reported no adverse events other than muscle soreness or joint complaints (low- to very low-certainty evidence). AUTHORS' CONCLUSIONS: The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no effect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with robust methodology and greater numbers of participants is still required.

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.

Abnormal lactate levels in patients with polymyositis and dermatomyositis: the benefits of a specific rehabilitative program.

BACKGROUND: Polymyositis (PM) and Dermatomyositis (DM) are chronic, inflammatory and autoimmune skeletal muscle disorders characterized by reduced muscle strength, fatigue and myalgia. While inflammation causes muscle damage in the early phase, metabolic alterations such as an impairment of oxidative metabolism seem to be responsible for the disability in the chronic phase of the disease. AIM: To assess muscle oxidative efficiency and to test the effect of aerobic training in a group of PM/DM patients. DESIGN: A case-control study and a within-group comparison. SETTING. Outpatients of the Unit of Neurorehabilitation of the University Hospital of Pisa. POPULATION: 20 patients with myositis (15 PM and 5 DM) and 15 healthy subjects as a control group. METHODS: The test consisted of an incremental, sub-maximal aerobic exercise on a treadmill; haematic lactate was assessed at rest and after 1', 5', 10' and 30' minutes from the end of the exercise. A within-group comparison was conducted on four of the PM patients (P group). They were subjected to six weeks aerobic training. Lactate curve and functional tests were assessed before and after the treatment. RESULTS: A precocious fatigability and significantly higher values of lactate at rest and after the exercise were observed in patients. In the P group mean lactate levels were significantly decreased after the treatment and an improvement of muscle performance was observed. CONCLUSION: Abnormal blood lactate levels suggested an impaired muscle oxidative efficiency in PM/DM patients. A specific aerobic training program reduced lactate levels and relieved fatigue symptoms in a within-group of four of the PM patients. CLINICAL REHABILITATION IMPACT: Such a specific aerobic training program could be introduced in everyday practice for the rehabilitative treatment of PM/DM patients.

Aerobic training in persons who have recovered from juvenile dermatomyositis.

A recent study has shown that 36 persons who had recovered from juvenile dermatomyositis (JDM) have on average an 18% decrease in maximal oxygen uptake. The objective of this study was to investigate the effect of a 12-week aerobic training program in this group, and assess whether aerobic training can normalize aerobic capacity to the expected level for age and gender. The patients participating in the study, one male and nine females (16-42 years of age), were in remission from JDM, defined as no clinical or biochemical evidence of disease activity and no medical treatment for 1 year. The patients had a median disease duration of 3.4 years (1.4-10.3), a median treatment duration of 2.4 years (0.4-9.3) and a median duration of remission of 7.0 years (1.2-30.0). Patients trained at home on a cycle ergometer for 12 weeks at a heart rate interval corresponding to 65% of their maximal oxygen uptake (VO(2max)). VO(2max) and maximal workload (W(max)) were determined before and after the 12-week training period through an incremental cycling test to exhaustion. The patients served as their own controls. Eight patients with JDM in remission completed the 12-week exercise program; one patient completed 9 weeks out of the 12-week program and one dropped out of the study. Training increased VO(2max) and W(max) by 26% and 30% (P < 0.001). Creatine kinase (CK) levels were normal pre-training and did not change with training, reflecting no muscle damage. We also found that at a given workload, heart rate was lowered significantly after the 12-week training period, indicating an improvement in cardiovascular fitness. This study shows that 12 weeks of moderate-intensity aerobic training is an effective and safe method to increase oxidative capacity and fitness in persons who have recovered from JDM. The results indicate that the low oxidative capacity in JDM patients in remission is reversible and can be improved. Thus, we recommend frequent aerobic training to be incorporated into supervised physiotherapy sessions in the treatment of JDM patients in remission.

Dermatomiositis asociada a cáncer de mama. Síndrome paraneoplásico infrecuente / Dermatomyositis associated with breast cancer. An infrequent paraneoplastic syndrome

La dermatomiositis es un síndrome paraneoplásico raro que se asocia al diagnóstico de diferentes tumores. El cáncer de mama es un tumor asociado de manera muy infrecuente con la dermatomiositis. Se presenta el caso de una mujer de 50 años que comenzó como una dermatomiositis y que fue diagnosticada de un cáncer de mama. El tratamiento quirúrgico del cáncer de mama supuso la desaparición de los síntomas de debilidad muscular y de las alteraciones cutáneas(AU)

Dermatomyositis is a rare paraneoplastic syndrome associated with several malignant tumors. The association with breast cancer is much less frequent. We report a 50-year-old woman who presented with dermatomyositis and was diagnosed with breast cancer. Surgical treatment of breast cancer improved the symptoms of muscular weakness and the cutaneous manifestations of dermatomyositis(AU)

Improved exercise performance and increased aerobic capacity after endurance training of patients with stable polymyositis and dermatomyositis.

INTRODUCTION: This randomized, controlled study on patients with polymyositis or dermatomyositis was based on three hypotheses: patients display impaired endurance due to reduced aerobic capacity and muscle weakness, endurance training improves their exercise performance by increasing the aerobic capacity, and endurance training has general beneficial effects on their health status. METHODS: In the first part of this study, we compared 23 patients with polymyositis or dermatomyositis with 12 age- and gender-matched healthy controls. A subgroup of patients were randomized to perform a 12-week endurance training program (exercise group, n = 9) or to a non-exercising control group (n = 6). We measured maximal oxygen uptake (VO2 max) and the associated power output during a progressive cycling test. Endurance was assessed as the cycling time to exhaustion at 65% of VO2 max. Lactate levels in the vastus lateralis muscle were measured with microdialysis. Mitochondrial function was assessed by measuring citrate synthase (CS) and ß-hydroxyacyl-CoA dehydrogenase (ß-HAD) activities in muscle biopsies. Clinical improvement was assessed according to the International Myositis Assessment and Clinical Studies Group (IMACS) improvement criteria. All assessors were blinded to the type of intervention (that is, training or control). RESULTS: Exercise performance and aerobic capacity were lower in patients than in healthy controls, whereas lactate levels at exhaustion were similar. Patients in the exercise group increased their cycling time, aerobic capacity and CS and ß-HAD activities, whereas lactate levels at exhaustion decreased. Six of nine patients in the exercise group met the IMACS improvement criteria. Patients in the control group did not show any consistent changes during the 12-week study. CONCLUSIONS: Polymyositis and dermatomyositis patients have impaired endurance, which could be improved by 12 weeks of endurance training. The clinical improvement corresponds to increases in aerobic capacity and muscle mitochondrial enzyme activities. The results emphasize the importance of endurance exercise in addition to immunosuppressive treatment of patients with polymyositis or dermatomyositis. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01184625.

Strength training and aerobic exercise training for muscle disease.

BACKGROUND: Strength training or aerobic exercise programmes might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004. OBJECTIVES: To examine the safety and efficacy of strength training and aerobic exercise training in people with a muscle disease. SEARCH METHODS: We searched the Cochrane Neuromuscular Disease Group Specialized Register (July 2012), CENTRAL (2012 Issue 3 of 4), MEDLINE (January 1946 to July 2012), EMBASE (January 1974 to July 2012), EMBASE Classic (1947 to 1973) and CINAHL (January 1982 to July 2012). SELECTION CRITERIA: Randomised or quasi-randomised controlled trials comparing strength training or aerobic exercise programmes, or both, to no training, and lasting at least six weeks, in people with a well-described diagnosis of a muscle disease.We did not use the reporting of specific outcomes as a study selection criterion. DATA COLLECTION AND ANALYSIS: Two authors independently assessed trial quality and extracted the data obtained from the full text-articles and from the original investigators. We collected adverse event data from included studies. MAIN RESULTS: We included five trials (170 participants). The first trial compared the effect of strength training versus no training in 36 people with myotonic dystrophy. The second trial compared aerobic exercise training versus no training in 14 people with polymyositis and dermatomyositis. The third trial compared strength training versus no training in a factorial trial that also compared albuterol with placebo, in 65 people with facioscapulohumeral muscular dystrophy (FSHD). The fourth trial compared combined strength training and aerobic exercise versus no training in 18 people with mitochondrial myopathy. The fifth trial compared combined strength training and aerobic exercise versus no training in 35 people with myotonic dystrophy type 1.In both myotonic dystrophy trials and the dermatomyositis and polymyositis trial there were no significant differences between training and non-training groups for primary and secondary outcome measures. The risk of bias of the strength training trial in myotonic dystrophy and the aerobic exercise trial in polymyositis and dermatomyositis was judged as uncertain, and for the combined strength training and aerobic exercise trial, the risk of bias was judged as adequate. In the FSHD trial, for which the risk of bias was judged as adequate, a +1.17 kg difference (95% confidence interval (CI) 0.18 to 2.16) in dynamic strength of elbow flexors in favour of the training group reached statistical significance. In the mitochondrial myopathy trial, there were no significant differences in dynamic strength measures between training and non-training groups. Exercise duration and distance cycled in a submaximal endurance test increased significantly in the training group compared to the control group. The differences in mean time and mean distance cycled till exhaustion between groups were 23.70 min (95% CI 2.63 to 44.77) and 9.70 km (95% CI 1.51 to 17.89), respectively. The risk of bias was judged as uncertain. In all trials, no adverse events were reported. AUTHORS' CONCLUSIONS: Moderate-intensity strength training in myotonic dystrophy and FSHD and aerobic exercise training in dermatomyositis and polymyositis and myotonic dystrophy type I appear to do no harm, but there is insufficient evidence to conclude that they offer benefit. In mitochondrial myopathy, aerobic exercise combined with strength training appears to be safe and may be effective in increasing submaximal endurance capacity. Limitations in the design of studies in other muscle diseases prevent more general conclusions in these disorders.

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.

OBJECTIVE: 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. METHODS: Patients were randomized into a 12-week endurance exercise program group (EG; n = 11) or a control group (CG; n = 10). Assessments of health (Short Form 36 [SF-36]), muscle performance (5 voluntary repetition maximum [5 VRM]), activities of daily living (ADL), patient preference (McMaster Toronto Arthritis Patient Preference Disability Questionnaire), VO2 max, and disease activity (International Myositis Assessment and Clinical Studies criteria of improvement of the 6-item core set) were performed at 0 and 12 weeks. Disability assessments were performed again at 52 weeks in an open extension period. All assessments were performed by blinded observers. RESULTS: The EG improved compared to the CG in SF-36 physical function and vitality (P = 0.010 and P = 0.046, respectively), ADL score (P = 0.035), 5 VRM (P = 0.026), and VO2 max (P = 0.010). More patients in the EG (7 of 11) were responders with reduced disease activity compared to none in the CG (P = 0.002). Correlations between VO2 max and SF-36 physical function were 0.90 and 0.91 at 0 and 12 weeks, respectively (P < 0.05). The EG improvement in 5 VRM was sustained up to 52 weeks compared to baseline (5.7 kg; P < 0.001), but not in ADL score or SF-36. CONCLUSIONS: Endurance exercise improves health and may reduce disease activity in patients with established PM/DM. This potentially could be mediated through improved aerobic fitness. The results also indicate sustained muscle strength up to 1 year after a supervised program.

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.

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)

The differential effect of child age on the illness intrusiveness--parent distress relationship in juvenile rheumatic disease.

OBJECTIVE: Examine age-related patterns of association between parent-reported illness intrusiveness and parent distress in parents of youth with juvenile rheumatic diseases (JRDs). STUDY DESIGN: Cross-sectional multiple regression analysis tested child age as a moderator in the illness intrusiveness-distress relationship. PARTICIPANTS: Fifty-two parents of children ages 9-17 diagnosed with JRD. MAIN OUTCOME MEASURES: The Illness Intrusiveness Scale--Parent Version and the Brief Symptom Inventory. RESULTS: Parent-reported illness intrusiveness demonstrated a significant main effect on distress for all parents in the sample. This was qualified by an Illness Intrusiveness x Child Age interaction. Illness intrusiveness was found to be significantly related to distress among parents of older youth, but was only marginally related to distress for parents of younger children. CONCLUSIONS: Results are consistent with family life cycle development models of adjustment to childhood chronic illness. The clinical implications of the findings are also discussed.

Exercise effects in patients with adult idiopathic inflammatory myopathies.

PURPOSE OF REVIEW: To give an update on recent findings on effects of exercise in patients with adult inflammatory myopathies. RECENT FINDINGS: Although responding to treatment, a majority of patients with polymyositis and dermatomyositis develop sustained disability. The reason for this is not clear. However, a recent study further supports the hypothesis of hypoxia in muscle tissue as a contributor to muscle weakness. The percentage of type I oxygen-dependent muscle fibers increased after a 12-week submaximal home exercise program along with improved muscle endurance in patients with chronic polymyositis or dermatomyositis. Creatine supplements in addition to the same home exercise program are more beneficial than exercise alone in patients with chronic polymyositis or dermatomyositis. Patients with chronic disease tolerate intensive resistance training resulting in improved muscle strength and muscle endurance. This 7-week exercise study also reported reduced disease activity and possibly even reduced muscle inflammation. SUMMARY: These recent studies are in line with earlier ones further supporting safety and efficacy of exercise in patients with polymyositis or dermatomyositis. There is an urgent need for larger randomized controlled trials also including patients with inclusion body myositis to further increase knowledge of disease mechanisms causing disability, exercise effects, and what exercise program is most efficient in patients with different entities of idiopathic inflammatory myopathies.

Effect of physical training on the proportion of slow-twitch type I muscle fibers, a novel nonimmune-mediated mechanism for muscle impairment in polymyositis or dermatomyositis.

OBJECTIVE: To compare muscle fiber type composition and muscle fiber area in patients with chronic polymyositis or dermatomyositis and healthy controls, and to determine whether physical training for 12 weeks could alter these muscle characteristics. METHODS: Muscle fiber type composition and muscle fiber area were investigated by biochemical and immunohistochemistry techniques in repeated muscle biopsy samples obtained from 9 patients with chronic myositis before and after a 12-week exercise program and in healthy controls. Muscle performance was evaluated by the Functional Index (FI) in myositis and by the Short Form 36 (SF-36) quality of life instrument. RESULTS: Before exercise, the proportion of type I fibers was lower (mean +/- SD 32% +/- 10%) and the proportion of type IIC fibers was higher (3% +/- 3%) in patients compared with healthy controls. After exercise, percentage of type I fiber increased to 42% +/- 13% (P < 0.05), and type IIC decreased to 1% +/- 1%. An exercise-induced 20% increase of the mean fiber area was also observed. The functional capacity measured by the FI in myositis and the physical functioning subscale of the SF-36 increased significantly. Improved physical functioning was positively correlated with the proportion of type I fibers (r = 0.88, P < 0.01) and type II muscle fiber area (r = 0.70, P < 0.05). CONCLUSION: Low muscle endurance in chronic polymyositis or dermatomyositis may be related to a low proportion of oxidative, slow-twitch type I fibers. Change in fiber type composition and increased muscle fiber area may contribute to improved muscle endurance and decreased muscle fatigue after a moderate physical training program.

Benefits of intensive resistance training in patients with chronic polymyositis or dermatomyositis.

OBJECTIVE: To investigate the benefits and safety of an intensive muscular training program in patients with chronic polymyositis (PM) and dermatomyositis (DM). METHODS: Nine patients with chronic PM or DM (median age 53 years, range 44-61) were included. Assessments of impairment (10-15 voluntary repetition maximum [VRM], the Functional Index 2 [FI-2], the Grippit, and pain rated on the Borg CR-10 scale), activity limitation (Myositis Activities Profile), and participation restriction (patients' disease impact on well-being) were performed 4 weeks prior to baseline, at baseline, and after 7 weeks of exercise. A 6-item core set of disease activity measures was administered and muscle biopsy samples of vastus lateralis were obtained at baseline and after 7 weeks of exercise. Response criteria at an individual level were set for disability and disease activity. The patients exercised 3 days per week for 7 weeks on loads allowing 10 VRM. RESULTS: On a group level there were no significant differences between assessments at 4 weeks before baseline compared with baseline. The group improved significantly regarding 10-15 VRM and FI-2 at 7 weeks compared with baseline (P < 0.05). All patients were responders with respect to impairment and 2 were activity limitation responders whereas participation restriction remained unchanged in all. Two patients were responders with reduced disease activity and no patient had signs of increased muscle inflammation in the muscle biopsy sample after 7 weeks of exercise. CONCLUSION: Patients with chronic, stable PM and DM can perform this intensive resistive exercise program with beneficial effects on impairment and activity limitation without increased muscle inflammation.

[Rehabilitation for myositis in acute phase].

Polymyositis and dermatomyositis (PM/DM) are representative inflammatory muscle diseases. In treatment of PM/DM, drug therapies are cardinal but rehabilitation may be another option. Since muscles with PM/DM are fragile for muscle exercise, rehabilitation has been recommended mainly in chronic phase. Some researchers considered that rehabilitation for PM/DM patients in acute phase may improve their functional prognosis without major sideeffect. However, there are controversies about rehabilitation for PM/DM patients from acute phase. To consider advisability, I reviewed literatures concerning rehabilitation for PM/DM patients in acute phase.

Advances in the treatment of juvenile dermatomyositis.

PURPOSE OF REVIEW: Juvenile dermatomyositis is a rare chronic inflammatory disease that primarily affects the muscles and skin. Immunosuppressive therapy has played a very important role in reducing mortality rates and morbidity. The review focuses on the spectrum of medications currently used in the treatment of juvenile dermatomyositis, highlighting new advances and unanswered questions. RECENT FINDINGS: Data regarding the treatment of juvenile dermatomyositis come almost entirely from retrospective studies with relatively small numbers of patients. Corticosteroids continue to be the accepted first-line therapy. Evidence that the addition of methotrexate at initiation of treatment allows corticosteroids to be tapered more rapidly with good outcomes exists. High-risk, refractory patients may benefit from intravenous cyclophosphamide. Results in refractory patients treated with rituximab are also encouraging. Topical immunosuppressant agents have been largely disappointing in treating rash. The effect and role of exercise in the treatment and rehabilitation of patients with juvenile dermatomyositis is an interesting new area of research. SUMMARY: Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity for the individual patient.

Functional outcome and quality of life in adult patients with idiopathic inflammatory myositis.

OBJECTIVES: To present the outcome of patients with idiopathic inflammatory myositis, focusing on functional ability and quality of life. METHODS: Analysis was performed using data from 105 adult patients with definitive polymyositis, dermatomyositis or overlap myositis, who were followed up at a single centre. The diagnosis was made between 1979 and 2000 based on Bohan and Peter's criteria. Functional ability was assessed after a minimum follow-up of 3 yr with the Health Assessment Questionnaire Disability Index (HAQDI) and quality of life was measured with the Short Form 36-item questionnaire (SF-36). RESULTS: Fifteen patients in our cohort died and 87 participated in the evaluation of functional outcome. Functional ability after a median follow-up of 107.1 months (range 36.4-273.3) was heterogeneous. The median HAQDI score was 0.875 (range 0-2.875). Polyphasic or chronic-progressive disease course, osteoporosis and long-term follow-up were predictive of higher HAQDI scores. In terms of quality of life, significant differences from population norms were shown in all domains of the SF-36. There were no significant differences in the SF-36 scores among the patients according to clinicopathological subset or disease course. CONCLUSIONS: Although the mortality of our cohort was favourable, myositis continues to have a great impact on life in the medium and long term. The present work indicates that myositis patients have a significantly poorer quality of life than the normal population, but there was no difference among the patients according to clinicopathological subsets.