Exercise and Inclusion Body Myositis (IBM).

Site presented by Bill Tillier

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⬛ Webpage Menu:

Page Menu:

⬛ 1. Overview.

⬛ 2. Key research findings.

⬛ 3. Major research issues.

⬛ 4. Patient experiences.

⬛ 5. References 2010 →

⬛ 6. References before 2010.

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1. Overview.

The overriding message in this webpage is safety first.

⬛ It seems increasingly recognized that an exercise program of some type, specifically customized for the patient, may be an important treatment component during the early phases of sIBM.

⬛ Is exercise safe in IBM? Based on today’s research, the answer is not yet clear, it probably is in many (or most?) cases, but not all: this likely depends on several factors.

⬛ Is exercise helpful in IBM? Based on today’s research, the answer is not yet clear, it probably is in many (or most?) cases, but not all: this likely depends on several factors.

⬛ Exercise temporarily increases CK levels and therefore, in the past, the conventional wisdom was that exercise should be avoided in patients with myositis conditions. In terms of IBM, many researchers are beginning to advocate for exercise programs, however, the literature emphasizes that more research needs to be done.

⬛ In the past it was suggested that exercise should focus on muscle groups that are not yet involved.

⬛ In my opinion, no exercise program should be started without the advice of your medical team and its direct supervision of what you are doing.

⬛ Again, in my opinion, great care must be used because overexercising diseased muscles could increase weakness. Patients often exercise without supervision, overexercise and then later report "overdoing it" and feeling an increase in weakness with little or no subsequent recovery.

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2. Key research findings.

⬛ "Clinical experience suggests a variation in exercise response in patients with inclusion body myositis. Again, diagnosis duration and degree of damage are two important factors, but there are probably different subtypes of inclusion body myositis with varying muscle involvement and progression time and knowledge of the roles of antibodies is very limited. Our own clinical experience indicates that most patients tolerate exercise well with limited improvements or mostly maintaining function. A few patients seem to respond very well with larger improvements in function and tolerating a very high exercise frequency and intensity, while yet a smaller group of patients might not tolerate even mild exercise. There is an urgent need for more, larger studies looking into exercise in inclusion body myositis" (Alexanderson, 2018, p. 295).

⬛ "No exercise recommendations are provided for patients with recent onset, active PM/DM, or IBM because there is only one RCT (randomized control trial) published for each of these patient subsets" (Alexanderson & Boström, 2020, p. 8).

⬛ "Despite the promising results reported in case studies and even clinical experience, the evidence sustaining physical training in sIBM is still fragile. Most studies are not controlled or randomized, and the heterogeneity of the training protocols makes it difficult to grasp the ideal intervention. Currently, there is limited evidence to funding the safety of physical exercise in sIBM. Based on the conflicting and low-quality evidence, physical exercise might slightly improve muscle strength or, at least, reduce the speed of decline" (de Souza et al., 2020, p. 5)

⬛ "Exercise is likely to form an increasingly important facet of management of patients with IBM, but the optimal type of exercise programme to enrol patients in is not yet determined" (Lilleker, 2018, p. 644).

⬛ "Good-quality evidence is needed so people with IBM can be confident their exercise prescription will lead to functional improvement with minimum adverse effects" (Machado et al., 2014, p. 477).

⬛ "We conclude that, in this patient, lingual [tongue] strengthening slowed the progression of disease-related lingual strength loss and extended functional swallowing performance. Thus, this type of intervention may hold promise as an effective swallowing treatment option for patients with neurodegenerative inflammatory diseases such as inclusion body myositis and Sjögren’s syndrome" (Malandraki et al., 2012, p. 1469).

⬛ "We have identified clinical subgroups within the IBM cohort, according to strength and functional status change over time. These findings may lead to enhanced clinical prognostication and potential stratification of trial participants (Oldroyd et al., 2020, p. 81).

⬛ "Physical therapy plays a significant role in the treatment of IBM, since it leads to improvement of the functional capacity of patients in daily activities, thus reducing their disability" (Stevanović et al., 2020, p. 1220).

⬛ Other implications of this research review:

▣ The idea that there may be subtypes of IBM is an important hypothesis that requires more research inquiry.

▣ One of the studies reviewed (Chinoy & Lilleker, 2020) indicated that IBM does not respond to immunosuppressive therapy while another (Dimachkie & Barohn, 2014) indicated immunosuppressant therapy may negatively impact patients with IBM.

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3. Major research issues.

⬛ It is very difficult to do this research because IBM is a very variable disease and different patients may be experiencing different levels of impact on different muscles.

⬛ Different studies generally use different exercise programs.

⬛ Some studies use a special type of exercise where blood flow is restricted. For example, a blood pressure cuff is put on the arm reducing the blood flow and then the wrist and hand are exercised. This is called Blood-flow restricted resistance training.

⬛ Measurement of strength is a major issue. What is the best way to measure changes?

⬛ Placebo effect: it is well recognized that about 30% of people will report strong positive results after any kind of treatment. Objective measurement of strength has shown that in some of these cases no change actually took place. This complicates research that use surveys that ask patients about their strength.

⬛ There are few people in the treatment community who have the knowledge and training to conduct or supervise exercise programs.

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4. Patient experiences.

⬛ Patient #1.

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5. References 2010 →

⬛ Alemo Munters, L., Alexanderson, H., Crofford, L. J., & Lundberg, I. E. (2014). New insights into the benefits of exercise for muscle health in patients with idiopathic inflammatory myositis. Current Rheumatology Reports, 16(7), 429. https://doi.org/10.1007/s11926-014-0429-4

⬛ Alexanderson, H. (2012). Exercise in Inflammatory Myopathies, Including Inclusion Body Myositis. Current Rheumatology Reports, 14(3), 244–251. https://doi.org/10.1007/s11926-012-0248-4

▣ "All together, four open studies and two case reports have evaluated safety and the effects of exercise in patients with sporadic inclusion body myositis" (p. 245).

⬛ Alexanderson, H. (2016). Physical exercise as a treatment for adult and juvenile myositis. Journal of Internal Medicine, 280(1), 75–96. https://doi.org/10.1111/joim.12481

⬛ Alexanderson, H. (2018). Exercise in myositis. Current Treatment Options in Rheumatology, 4(4), 289–298. https://doi.org/10.1007/s40674-018-0113-3

▣ "Clinical experience suggests a variation in exercise response in patients with inclusion body myositis. Again, diagnosis duration and degree of damage are two important factors, but there are probably different subtypes of inclusion body myositis with varying muscle involvement and progression time and knowledge of the roles of antibodies is very limited. Our own clinical experience indicates that most patients tolerate exercise well with limited improvements or mostly maintaining function. A few patients seem to respond very well with larger improvements in function and tolerating a very high exercise frequency and intensity, while yet a smaller group of patients might not tolerate even mild exercise. There is an urgent need for more, larger studies looking into exercise in inclusion body myositis" (p. 295).

⬛ Alexanderson, H., & Boström, C. (2020). Exercise therapy in patients with idiopathic inflammatory myopathies and systemic lupus erythematosus – A systematic literature review. Best Practice & Research Clinical Rheumatology, 34(2), https://doi.org/10.1016/j.berh.2020.101547

▣ "There is insufficient evidence for effects of exercise in patients with recent onset PM/DM and IBM" (p. 2).

▣ "The natural course of IBM involves a steady decline in muscle strength by about 10% per year [29]" (p. 3).

▣ "The literature also suggest that exercise can improve aerobic capacity, muscle function, and quality of life in patients with established PM/DM, while results are diverging in patients with recent onset, active PM/DM, and in patients with IBM" (p. 4).

▣ "No exercise recommendations are provided for patients with recent onset, active PM/DM, or IBM because there is only one RCT published for each of these patient subsets" (p. 8).

⬛ Alexanderson, H., & Regardt, M. (2020). Role of exercise in the management of myositis. In Managing Myositis (pp. 323–334). Springer International Publishing. https://doi.org/10.1007/978-3-030-15820-0_33

⬛ Chinoy, H., & Lilleker, J. B. (2020). Pitfalls in the diagnosis of myositis. Best Practice & Research Clinical Rheumatology, 34(1), 101486. https://doi.org/10.1016/j.berh.2020.101486

▣  "IBM does not respond to treatment with immunosuppressive treatment, which should be avoided. A diagnosis of IBM should be alerted to when patients prove refractory to treatment" (p. 9).

⬛ Clarkson, M. J., May, A. K., & Warmington, S. A. (2020). Is there rationale for the cuff pressures prescribed for blood flow restriction exercise? A systematic review. Scandinavian Journal of Medicine & Science in Sports, 30(8), 1318–1336. https://doi.org/10.1111/sms.13676

⬛ Connor, S., & Needham, M. (2021). A double-blinded randomised controlled trial to establish whether testosterone treatment combined with exercise improves muscle strength and function and quality of life in men affected by inclusion body myositis. Tasman Medical Journal, 3(1), 69–75.

⬛ de Souza, F. C., Behrens Pinto, G., de Souza, J., Olivo Pallo, P., Hoff, L., & Shinjo, S. (2020). Sporadic inclusion body myositis in the rheumatology clinic. Indian Journal of Rheumatology, 15(6), 145. https://doi.org/10.4103/injr.injr_80_20

▣ Although there have been no robust clinical trials, training exercise/physiotherapy should be prescribed regularly in sIBM patients.

▣ "Despite the promising results reported in case studies and even clinical experience, the evidence sustaining physical training in sIBM is still fragile. Most studies are not controlled or randomized, and the heterogeneity of the training protocols makes it difficult to grasp the ideal intervention. Currently, there is limited evidence to funding the safety of physical exercise in sIBM. Based on the conflicting and low-quality evidence, physical exercise might slightly improve muscle strength or, at least, reduce the speed of decline" (p. 5).

⬛ Dimachkie, M. M., & Barohn, R. J. (2014). Inclusion Body Myositis. Neurologic Clinics, 32(3), 629–646.https://doi.org/10.1016/j.ncl.2014.04.001

▣ "in a long-term observational study of 136 patients, those who received immunosuppressive treatments (52%) were more severely affected on disability scales and on the sporadic IBM (sIBM) weakness composite index compared with those who did not.[52] Progression toward walking handicap was more rapid among patients receiving immunosuppressive treatments (p. 638).

▣ "There is a role for physical therapy, orthotic devices, occupational therapy, a healthy well-balanced diet, and exercise in IBM. Despite falls being a common occurrence for people with IBM, falls guidelines are not being followed, and referral rates to physiotherapy need to improve.[13] A tailored 12-week home exercise program, 5 days a week for 12 weeks, in combination with stationary biking or walks was safe in 7 patients.75 There was no strength deterioration, no change in serum CK, and no increase in muscle inflammation on biopsy. However, the study was not able to show improved muscle strength or function."(p. 642).

▣ "Given the encouraging safety data, we recommend to our patients with IBM nonfatiguing exercises of mild to moderate-intensity. There is a suggestion that exercise might lead to modestly improved or sustained muscle strength in some patients. However, there are conflicting data on the effect of exercise on the 2 muscle groups most severely affected in IBM (finger flexors and knee extensors) and on the potential for functional mobility benefit. Large multicenter controlled trials are needed to clarify any potential gains from exercise in people with IBM" (p. 642).

⬛ Gualano, B., Ugrinowitsch, C., Neves Jr., M., Lima, F. R., Pinto, A. L. S., Laurentino, G., Tricoli, V. A. A., Lancha Jr., A. H., & Roschel, H. (2010). Vascular Occlusion Training for Inclusion Body Myositis: A Novel Therapeutic Approach. Journal of Visualized Experiments, 40, 1894.https://doi.org/10.3791/1894

▣ "Resistance training with vascular occlusion is a relatively novel training method, but results are very encouraging. We have found that after a 12-wk training program, an IBM patient's leg press one-repetition maximum, balance and mobility function, and thigh cross-sectional area increased 15.9%, 60%, and 4.7%, respectively" (p. 1).

⬛ Gupta, A. D., & Quadros, N. (2009). Intensive rehabilitation in a patient with inclusion body myositis. Medical Journal of Australia, 190(4), 208–209. https://doi.org/10.5694/j.1326-5377.2009.tb02352.x

▣ "A specifically tailored rehabilitation program improved our patient’s physical function without evidence of increased muscle damage. … We believe that the option of active rehabilitation should always be explored, regardless of the apparent severity of underlying medical conditions" (p. 208).

⬛ Harrigfeld, T., & Jackman, T. (2017). Outpatient Physical therapy evaluation and treatment of a patient diagnosed with sporadic inclusion body myositis: A case study. International Journal of Physiotherapy, 236–240.https://doi.org/10.15621/ijphy/2017/v4i4/15472

▣ "Case Summary: The patient was a 66-year-old male who was diagnosed with sporadic inclusion body myositis with a chief complaint of weakness and fall risk. He presented with generalized lower extremity weakness and atrophy of bilateral quadriceps, as well as impaired balance and increasing fatigue with activity. Therapeutic exercise, home exercise program, balance, gait, and stair training were delivered to address these impairments. Patient outcomes showed improvement in balance and safety with functional activities" (p. 236).

▣ "Discussion: The patient was seen for seven visits that were 45 – 60 minutes in length, over a five-week period. The patient made subjective reports of improvement in functional activities and balance; however many objective outcome measures could not be reassessed. There is a need for further research on this population to determine the effectiveness and parameters of physical therapy interventions." (p. 236).

▣ "Conclusion: Physical therapy may have helped improve balance as well as subjective reports from the patient of increased feeling of confidence while navigating stairs." (p. 236).

⬛ Johnson, L. G., Edwards, D. J., Walters, S., Thickbroom, G. W., & Mastaglia, F. L. (2007). The Effectiveness of an Individualized, Home-Based Functional Exercise Program for Patients With Sporadic Inclusion Body Myositis: Journal of Clinical Neuromuscular Disease, 8(4), 187–194.https://doi.org/10.1097/CND.0b013e3181237291

▣ "Because of the variability in the degree of weakness, level of endurance, and general level of fitness among patients with IBM, it is important that any exercise program should be designed for the individual, that the initial exercise load should not be excessive, and that the exercise program should be incremental and take into account not only gains in strength but also the overall functional capacity of the patient" (p. 188).

▣ "studies with patients with IBM using strength and aerobic training concluded that exercise can be performed safely, can lead to dynamic strength improvements, and possibly can prevent continued loss of muscle strength.[15,16] However, optimal exercise programs resulting in improvements in muscle strength and function have not been established in either a home-based or clinic setting. The emphasis of this study on selecting a set of exercises designed to improve limb and whole-body function, including the use of multijoint exercises and the integration of balance and stabilizing components, distinguishes this study from previous exercise intervention studies in IBM. This study has shown that a closely monitored, 16-week, home-based, individualized functional exercise program can lead to significant gains in muscle strength and improvements in the performance of functional tasks in patients with IBM" (p. 192).

▣ " In conclusion, we have shown that an individualized, supervised program of mild daily exercises performed in the patient’s own home can lead to significant increases in muscle strength and to useful functional improvements in walking and mobility in patients with IBM, even in those with longstanding disease. Such changes have positive implications for the patients’ overall wellbeing and capacity to perform activities of daily living" (p. 193).

⬛ Johnson, L. G., Collier, K. E., Edwards, D. J., Philippe, D. L., Eastwood, P. R., Walters, S., Thickbroom, G. W., & Mastaglia, F. L. (2009). Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis. Journal of Clinical Neuromuscular Disease, 10(4), 178–184.https://doi.org/10.1097/CND.0b013e3181a23c86

▣ Methods: Aerobic capacity, muscle strength, and functional capacity assessments were conducted on 7 participants with sporadic IBM before and after a 12-week exercise program, which included resistance exercises and aerobic stationary cycling 3 times per week on alternative days.

▣ Results: Aerobic capacity of the group increased significantly by 38%, and significant strength improvements were observed in 4 of the muscle groups tested (P less than 0.05). The exercise program was well tolerated, and there was no significant change in the serum creatine kinase level after the exercise period.

▣ Conclusions: An aerobic exercise program can be safely tolerated by patients with sporadic IBM and can improve aerobic capacity and muscle strength when combined with resistance training. These findings indicate that aerobic and functional muscle strengthening exercise should be considered in the management of patients with IBM.

▣ "In summary, this study has shown that significant improvements in aerobic capacity can be attained through a home-based, patient-specific functional and aerobic exercise program. Furthermore, in the absence of muscle damage, functional improvements can be made and significant strength gains can be attained in nondiseased muscles when trained. The results of this study indicate that patients with IBM can improve their aerobic capacity with training; however, the selective nature of this disease may restrict the potential strength gains in muscles involved in IBM, and it remains to be seen if exercise can produce long term disease-modifying benefits. Importantly, the training program was safe and tolerable. The findings from this study suggest that aerobic and functional muscle strengthening exercises are complementary and should be considered in the management of patients with IBM" (p. 183).

⬛ Jørgensen, A. N., Aagaard, P., Frandsen, U., Boyle, E., & Diederichsen, L. P. (2018). Blood-flow restricted resistance training in patients with sporadic inclusion body myositis: A randomized controlled trial. Scandinavian Journal of Rheumatology, 47(5), 400–409.https://doi.org/10.1080/03009742.2017.1423109

▣ "These findings indicate that BFR training may have provided a protective effect on mechanical muscle function in the present group of sIBM patients, helping to minimize the long-term progressive loss of contractile muscle properties caused by the disease" (p. 406).

⬛ Jørgensen, A. N., Aagaard, P., Nielsen, J. L., Christiansen, M., Hvid, L. G., Frandsen, U., & Diederichsen, L. P. (2017). Physical function and muscle strength in sporadic inclusion body myositis. Muscle & Nerve, 56(6), E50–E58. https://doi.org/10.1002/mus.25603

▣ "In this study we examined the link between health-related quality of life, functional capacity, and mechanical muscle function in sIBM patients. The main findings of this study are that TUG test [timed up & go test (TUG)], performance was the strongest predictor of self-reported physical function, and that interlimb maximal knee extensor muscle strength asymmetry was the strongest predictor of horizontal gait function in this group of sIBM patients" (p. e54).

⬛ Jørgensen, A. N., Aagaard, P., Nielsen, J. L., Frandsen, U., & Diederichsen, L. P. (2016). Effects of blood-flow-restricted resistance training on muscle function in a 74-year-old male with sporadic inclusion body myositis: A case report. Clinical Physiology and Functional Imaging, 36(6), 504–509.https://doi.org/10.1111/cpf.12259

▣ "The objective was to investigate the effect of low-load resistance exercise with concurrent partial blood flow restriction to the working muscles (blood-flow-restricted (BFR) training) in a patient with sIBM. The training consisted of 12 weeks of lower extremity BFR training with low training loads (~25-RM). The patient was tested for mechanical muscle function and functional capacity before and after 6 and 12 weeks of training. Maximal horizontal gait speed increased by 19%, which was accompanied by 38–92% improvements in mechanical muscle function (maximal isometric strength, rate of force development and muscle power). In conclusion, BFR training was well tolerated by the patient with sIBM and led to substantial improvements in mechanical muscle function and gait speed" (p. 504).

⬛ Korotkova, M., Sandström, S., Hallén, A., Idborg, H., Dorph, C., & Alexanderson, H. (2016). A7.22 Effects of whole-body vibration exercise on physical capacity and prostaglandin metabolites in patients with sporadic inclusion body myositis. Annals of the Rheumatic Diseases, 75(Suppl 1), A64–A65. https://doi.org/10.1136/annrheumdis-2016-209124.153

▣ Objective To evaluate the effects of whole-body vibration (WBV) exercise on physical capacity, disease activity and urine levels of prostaglandin metabolites (PG-M) in patients with sIBM.

▣ Conclusions The WBV exercise resulted in improved physical capacity. This program was safe with stable prostaglandin and CK levels throughout the study. One P even had reduced disease activity.

⬛ Lilleker, J. B. (2018). Advances in the early diagnosis and therapy of inclusion body myositis: Current Opinion in Rheumatology, 30(6), 644–649. https://doi.org/10.1097/BOR.0000000000000537

▣ "exercise regimens come in various shapes and sizes, with little to guide the clinician as to which type of intervention might be most helpful in IBM" (p. 648).

▣ "Exercise is likely to increasingly become a key facet in the management of IBM and may require reorganization of clinics to ensure the regular engagement of the relevant physical therapists" (p. 648).

⬛ Machado, P. M., Ahmed, M., Brady, S., Gang, Q., Healy, E., Morrow, J. M., Wallace, A. C., Dewar, L., Ramdharry, G., Parton, M., Holton, J. L., Houlden, H., Greensmith, L., & Hanna, M. G. (2014). Ongoing Developments in Sporadic Inclusion Body Myositis. Current Rheumatology Reports, 16(12), 477.https://doi.org/10.1007/s11926-014-0477-9

▣ Although historically people with muscle conditions were advised not to exercise for fear of increasing damage, in recent years several small non-randomised studies have revealed benefits of exercise in neuromuscular conditions" (p. 8).

▣ "For people with IBM, moderate-intensity strength training is established as safe (by serum markers of inflammation and muscle biopsy), with mixed reports on efficacy" (p. 8).

▣ "Unfortunately, increasing evidence for the safety and efficacy of exercise has not reached the patient population. A recent study found they spent less time exercising and reported more barriers to exercise than controls. The most common barriers were lack of energy and motivation, and concerns about health, pain, and accelerating the degenerative process.[142] Good-quality evidence is needed so people with IBM can be confident their exercise prescription will lead to functional improvement with minimum adverse effects" (p. 8).

⬛ Malandraki, G. A., Kaufman, A., Hind, J., Ennis, S., Gangnon, R., Waclawik, A., & Robbins, J. (2012). The Effects of Lingual Intervention in a Patient With Inclusion Body Myositis and Sjögren’s Syndrome: A Longitudinal Case Study. Archives of Physical Medicine and Rehabilitation, 93(8), 1469–1475. https://doi.org/10.1016/j.apmr.2012.02.010

▣ "Participant: The subject was a 77-year-old woman who was diagnosed with inclusion body myositis and Sjögren’s syndrome … Intervention: The patient participated in an intensive 8-week lingual strengthening protocol 3 times (at years 1, 4, and 5) and a subsequent maintenance program twice (at years 4 and 5). … Main Outcome Measures: Three outcome measures were collected during the study: (1) lingual manometric pressures at the anterior and posterior tongue, measured by using a lingual manometric device, (2) airway invasion measured by using an 8-point Penetration-Aspiration Scale, and (3) clearance of the bolus measured by using a 3-point residue scale. … Results: Isometric lingual strengthening was effective in maintaining posterior tongue lingual pressure and Penetration-Aspiration Scale scores during the treatment periods. Residue scale scores did not significantly change during treatment. … Conclusions: We conclude that, in this patient, lingual strengthening slowed the progression of disease-related lingual strength loss and extended functional swallowing performance. Thus, this type of intervention may hold promise as an effective swallowing treatment option for patients with neurodegenerative inflammatory diseases such as inclusion body myositis and Sjögren’s syndrome" (p. 1469).

⬛ Mohannak, N., Pattison, G., Radich, B., Hird, K., Godecke, E., Mastaglia, F., & Needham, M. (2020). Exploring the efficacy of the expiratory muscle strength trainer to improve swallowing in inclusion body myositis: A pilot study. Neuromuscular Disorders, 30(4), 294–300.https://doi.org/10.1016/j.nmd.2020.02.010

▣ "This pilot study recruited IBM patients with abnormal upper esophageal sphincter function and dysphagia into an exercise program. It was hypothesized that regular practice using the Expiratory Muscle Strength Trainer (EMST) device would improve hyolaryngeal movement by strengthening suprahyoid musculature and facilitate opening of the upper esophageal sphincter thereby improving swallowing and quality of life. Overall, IBM patients who used the EMST device demonstrated no improvement in swallowing function. Consistent with that result, there was also no change in measures of quality of life" (p. 294).

⬛ Oldroyd, A. G. S., Lilleker, J. B., Williams, J., Chinoy, H., & Miller, J. A. L. (2020). Long-term strength and functional status in inclusion body myositis and identification of trajectory subgroups. Muscle & Nerve, 62(1), 76–82.https://doi.org/10.1002/mus.26859

▣ "This study has quantified annual dynamometry-derived muscle strength and functional status change in a large 'real-world' IBM cohort with long follow-up duration and also identified distinct subgroups, according to change of grip and knee extension strength and functional status change over time" (p. 80).

▣ "In clinical practice, it is evident that not all patients with IBM follow a similar trajectory of disease progression; our study attempted to define whether there is a continuous spectrum of severity or whether patients congregate into discrete subgroups. The variation of progression among trajectory subgroups was marked" (p. 80).

▣ " Our identification of IBM subgroups, according to longitudinal strength and functional status change, may allow for more focused eligibility criteria for clinical trials reducing baseline heterogeneity among treatment and placebo groups … We have identified clinical subgroups within the IBM cohort, according to strength and functional status change over time. These findings may lead to enhanced clinical prognostication and potential stratification of trial participants (p. 81).

⬛ Paltiel, A. D., Ingvarsson, E., Lee, D. K. K., Leff, R. L., Nowak, R. J., Petschke, K. D., Richards-Shubik, S., Zhou, A., Shubik, M., & O’Connor, K. C. (2015). Demographic and clinical features of inclusion body myositis in North America: Inclusion Body Myositis Survey. Muscle & Nerve, 52(4), 527–533. https://doi.org/10.1002/mus.24562

▣ Factors Correlated with Function and Disability. A functional index was used to determine whether disability was associated with demographics and daily living. There was an association between age and functional ability (P less than 0.0001), indicating that older respondents experienced greater disability (Fig. 1A). Similarly, for every year since diagnosis, there was an average decrease of 0.38 in the overall functional index (P less than 0.0001), highlighting disease progression and impairment (Fig. 1B). We also found associations between exercise and degree of function (Fig. 2) by comparing the functional index of subjects who exercised (controlling for age and gender) to that of subjects who did not. We observed that participation in exercise was significantly associated (P less than 0.0001) with increased functional ability (Fig. 2A). Subjects who participated in swimming or other unspecified forms of exercise reported greater functional ability (Fig. 2B) than respondents who did not exercise (P = 0.0263 for swimming and P less than 0.0001 for unspecified forms). Participation in physical therapy was not associated significantly (P = 0.5042) with increased functional ability (Fig. 2B). The amount of time spent exercising was associated significantly with increased functional ability. Respondents who spent either up to 5 h or more than 5 h per week had significantly higher functional index scores (P less than 0.0001 for both categories) compared with respondents who did not exercise (Fig. 2C). Moreover, respondents who spent more than 5 h per week participating in exercise had greater functional ability (P = 0.0028) than those whose exercise was limited to 5 h or less (Fig. 2C). (Paltiel, 2015, p. 2).

⬛ Patterson, S. D., Hughes, L., Warmington, S., Burr, J., Scott, B. R., Owens, J., Abe, T., Nielsen, J. L., Libardi, C. A., Laurentino, G., Neto, G. R., Brandner, C., Martin-Hernandez, J., & Loenneke, J. (2019). Blood flow restriction exercise position stand: Considerations of methodology, application, and safety. Frontiers in Physiology, 10(MAY), 1–15.https://doi.org/10.3389/fphys.2019.00533

⬛ Santos, A. R., Neves, M. T., Gualano, B., Laurentino, G. C., Lancha, A. H., Ugrinowitsch, C., Lima, F. R., & Aoki, M. S. (2014). Blood flow restricted resistance training attenuates myostatin gene expression in a patient with inclusion body myositis. Biology of Sport, 31(2), 121–124. https://doi.org/10.5604/20831862.1097479

▣ "Objective: The aim of this study was to evaluate the effects of a resistance training programme on the expression of genes related to myostatin (MSTN) signalling in one inclusion body myositis patient. Methods: A 65-year-old man with inclusion body myositis underwent blood flow restricted resistance training for 12 weeks. The gene expression of MSTN, follistatin, follistatin-like 3, activin II B receptor, SMAD-7, MyoD, FOXO-3, and MURF-2 was quantified. Results: After 12 weeks of training, a decrease (25%) in MSTN mRNA level was observed, whereas follistatin and follistatin-like 3 gene expression increased by 40% and 70%, respectively. SMAD-7 mRNA level was augmented (20%). FOXO-3 and MURF-2 gene expression increased by 40% and 20%, respectively. No change was observed in activin II B receptor or MyoD gene expression. Conclusions: Blood flow restricted resistance training attenuated MSTN gene expression and also increased expression of myostatin endogenous inhibitors. Blood flow restricted resistance training evoked changes in the expression of genes related to MSTN signalling pathway that could in part explain the muscle hypertrophy previously observed in a patient with inclusion body myositis" (p. 121).

⬛ Snedden, A. M., Lilleker, J. B., & Chinoy, H. (2021). In pursuit of an effective treatment: The past, present and future of clinical trials in inclusion body myositis. Current Treatment Options in Rheumatology. https://doi.org/10.1007/s40674-020-00169-4

▣ "These studies confirm that exercise is safe in IBM patients, with objective improvements in strength being demonstrated. No improvement in mobility has been shown. However, as exercise has many physical and psychological benefits, most clinicians would refer to a physiotherapist for an exercise programme or recommend that patients with IBM regularly take part in moderate physical activity."

⬛ Stevanovic, J., Vulovic, M., Pavicevic, D., Bezmarevic, M., Stojkovic, A., Radunovic, A., Aksic, M., Milosevic, B., Cvetkovic, A., Jovanovic, M., & Ivosevic, A. (2020). Physical therapy improves motion in a patient with inclusion body myositis - a case report. Vojnosanitetski Pregled, 77(11), 1216–1220. https://doi.org/10.2298/VSP171110165S

▣ "At first, the application of physical therapy in IBM was considered contraindicated due to possible increase of inflammatory process in muscles, but later studies pointed to its significance in muscle strength improvement, endurance and increase of functional abilities of the patients related to movement [1,15]." (Stevanović, 2020, p. 1219).

▣ " Physical therapy plays a significant role in the treatment of IBM, since it leads to improvement of the functional capacity of patients in daily activities, thus reducing their disability" (p. 1220).

⬛ Suzuki, N., Soga, T., Izumi, R., Toyoshima, M., Shibasaki, M., Sato, I., Kudo, Y., Aoki, M., & Kato, M. (2020). Hybrid Assistive Limb® for sporadic inclusion body myositis: A case series. Journal of Clinical Neuroscience, 81, 92–94. https://doi.org/10.1016/j.jocn.2020.09.031

▣ We evaluated the efficacy of rehabilitation therapy with Hybrid Assistive Limb ® (HAL; hereafter HAL therapy) in three patients diagnosed with sporadic inclusion body myositis (sIBM) who were hospitalized to undergo HAL therapy. Among them, one patient participated in eight courses and the other two in two courses of HAL therapy between 2017 and 2020. We determined the mean rate of improvement in two minute walking distance and 6m walking speed at the time of hospital discharge. After HAL therapy, we confirmed the patients’ desire to continue the use of HAL. In one patient, we observed improvements of 146.0% and 120.0% in two-minute walk and 6 m walking speed, respectively, after the fist course of HAL therapy; these values are 133.7% and 130% after the eighth course of HAL therapy. These values exceeded 90% in the other two patients after the second course of HAL therapy. HAL therapy maintained both quantity and quality of ambulation and showed positive psychological effects on patient conditions because it reduces exercise load and facilitates safety. While HAL therapy might be effective in maintaining and improving ambulation in patients with sIBM, we should consider to discontinue HAL therapy as it increased risk of falling (p. 94).

⬛ Vencovský, J., Alexanderson, H., & Lundberg, I. E. (2019). Idiopathic inflammatory myopathies. Rheumatic Disease Clinics of North America, 45(4), 569–581. https://doi.org/10.1016/j.rdc.2019.07.006

▣ "It is important to assess physical capacity (muscle function and aerobic capacity) before initiation exercise and a first follow-up is relevant after approximately 12 weeks. Patients with severe ILD, severe pain, or patients with IBM might benefit from more frequent follow-ups for safety and optimization of exercise dose. To be effective, exercise needs to be performed with a certain intensity, frequency, and duration. Exercise should be initiated on a moderate level adapted to individual physical capacity" (pp. 577-578).

⬛ Wernbom, M., Schoenfeld, B. J., Paulsen, G., Bjørnsen, T., Cumming, K. T., Aagaard, P., Clark, B. C., & Raastad, T. (2020). Commentary: Can Blood Flow Restricted Exercise Cause Muscle Damage? Commentary on Blood Flow Restriction Exercise: Considerations of Methodology, Application, and Safety. Frontiers in Physiology, 11(March). https://doi.org/10.3389/fphys.2020.00243

▣ Objective The aim of this phase 2 trial was to ascertain the feasibility and effect of community-based aerobic exercise training for people with 2 of the more common neuromuscular diseases: Charcot-Marie-Tooth disease type 1A (CMT) and inclusion body myositis (IBM).
Methods A randomized single-blinded crossover trial design was used to compare a 12-week aerobic training program using recombinant exercise bicycles compared to a control period. The training occurred 3 times per week in community gyms local to the participants. Support was available from trained gym staff and a research physiotherapist. The 2 disease groups were analyzed separately. The primary outcome measure was peak oxygen uptake (VO 2 peak) during a maximal exercise test, with secondary measures of muscle strength, function, and patient reported measures.
Results Data from 23 people with CMT and 17 people with IBM were included in the analysis. Both disease groups had high levels of participation and demonstrated improvements in VO 2 peak, with a moderate effect size in the CMT participants (Cohen d = 0.53) and a strong effect size in the IBM group (Cohen d = 1.72). No major changes were observed in the secondary outcome measures. Qualitative interviews revealed that participants valued the support of gym instructors and the research physiotherapists in overcoming challenges to participation.
Conclusion Twelve weeks of aerobic training in community gyms was feasible, safe, and improved aerobic capacity in people with CMT and IBM.
Classification of evidence This study provides Class II evidence that for patients with CMT type 1A and IBM, an aerobic training program increases aerobic capacity.

⬛ Wallace, A., Pietrusz, A., Dewar, E., Dudziec, M., Jones, K., Hennis, P., Sterr, A., Baio, G., Machado, P. M., Laurá, M., Skorupinska, I., Skorupinska, M., Butcher, K., Trenell, M., Reilly, M. M., Hanna, M. G., & Ramdharry, G. M. (2019). Community exercise is feasible for neuromuscular diseases and can improve aerobic capacity. Neurology, 92(15), e1773–e1785. https://doi.org/10.1212/WNL.0000000000007265

⬛ Williams, V., Coles, T., Gnanasakthy, A., Demuro, C., Yarr, S., Williams, N., Lowes, L., Alfano, L., & Tseng, B. (2016). Psychometric validation of a patient-reported measure of physical functioning in sporadic inclusion body myositis. Muscle & Nerve, 54(4), 658–665.https://doi.org/10.1002/mus.25080

▣ "The Inclusion Body Myositis–Functional Rating Scale (IBM-FRS) is a disease-specific patient-reported outcome (PRO) measure that was adapted from the Amyotrophic Lateral Sclerosis Functional Rating Scale, an assessment of activities of daily living for ALS patients. Although it has been administered in studies with patients with sIBM, the IBM-FRS was not developed in accordance with the United States Food and Drug Administration (FDA) guidance Patient-Reported Outcome Measures: Use in Medical Product Development to Support Labeling Claims (PRO) … To address this gap and the unique experiences of patients with sIBM, the sIBM Physical Functioning Assessment (sIFA) was developed to assess the clinical progression and impact of sIBM on physical functioning and the potential benefit of new therapies in a clinical trial setting" (p. 658).

▣ "The sIFA is the only sIBM-specific measure that has been developed in alignment with the FDA Guidance specifically for use in clinical trials of sIBM therapies. In conjunction with objective measures of physical functioning, we also consider that the sIFA may be used in clinical practice to help monitor patient change. The psychometric results corroborated the findings from the qualitative research undertaken in the development of the sIFA. Collectively, the results indicate that the sIFA is a reliable, valid, useful, and appropriate measure of symptom status and physical functioning in patients with sIBM. We recommend continued monitoring of the psychometric properties of the sIFA in larger, future studies, with particular attention to the definition of responder using an anchor-based method that can accommodate the slow progression of sIBM" (p. 664).

▣ Zhang, H., Liu, Y., Ma, J., & Zheng, L. (2021). A systematic review of physical exercise for patients with idiopathic inflammatory myopathies. Nursing & Health Sciences, August, nhs.12817. https://doi.org/10.1111/nhs.12817

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6. References before 2010.


PubMed Link

Curr Opin Rheumatol. 2009 Mar;21(2):158-63.
Exercise effects in patients with adult idiopathic inflammatory myopathies.
Alexanderson H.
Department of Physical Therapy, Karolinska University Hospital, Stockholm, Sweden. helene.alexanderson@karolinska.se
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. PMID: 19339927

Current Rheumatology Reports 2005, 7: 115-124.

Exercise: An Important Component of Treatment in the Idiopathic Inflammatory Myopathies.

Alexanderson H. See pdf here.


Curr Opin Rheumatol. 2005 Mar;17(2):164-71.

The role of exercise in the rehabilitation of idiopathic inflammatory myopathies.

Alexanderson H, Lundberg IE.

Department of Physical Therapy, Rheumatology Unit, Karolinska University Hospital, Solna, Stockholm, Sweden, and Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.

PURPOSE OF REVIEW: The objective of this review is to provide an update on exercise and clinical assessment in the idiopathic inflammatory myopathies.
RECENT FINDINGS: Polymyositis, dermatomyositis and inclusion body myositis are rare conditions with muscle weakness as a common prominent feature. Earlier, these patients were discouraged from active exercise due to a fear of increased muscle inflammation with recommendations to rest, perform range of motion exercises and in some cases, isometric exercises. However, beginning in the 1990s, studies reported reduced disability in patients with chronic polymyositis/dermatomyositis following resistive mild/moderate to intensive muscular training and aerobic endurance training, without signs of increased muscle inflammation. Patients with active, recent onset disease seem to benefit from mild/moderate muscular exercise without signs of increased muscle inflammation. There is no evidence of increased muscle inflammation following exercise in inclusion body myositis. However the beneficial effects are unclear as one study report increased muscle strength, while the other could not achieve impairment reduction.
SUMMARY: Studies evaluating active exercise in IIM support the notion of safety and benefits. However, large multi-center studies are needed to fully establish the safety and benefits of different types of exercise. Data indicate that active exercise, adapted to disease activity and disability should be included in the rehabilitation of patients in all stages of IIM. The newly developed and validated outcome measures for patients with polymyositis and dermatomyositis help assess the effects of interventions on disease activity and disability in clinical trials and in clinical practice. However, there are no sensitive and valid outcome measure for patients with inclusion body myositis.


Rheumatology (Oxford). 2004 Jan;43(1):49-54. Epub 2003 Jul 16.

International consensus outcome measures for patients with idiopathic inflammatory myopathies. Development and initial validation of myositis activity and damage indices in patients with adult onset disease.

Isenberg DA, Allen E, Farewell V, Ehrenstein MR, Hanna MG, Lundberg IE, Oddis C, Pilkington C, Plotz P, Scott D, Vencovsky J, Cooper R, Rider L, Miller F; International Myositis and Clinical Studies Group (IMACS).

Center for Rheumatology, Department of Medicine, The Middlesex Hospital, University College London, London, UK. d.isenberg@ucl.ac.uk

OBJECTIVE: To devise new tools to assess activity and damage in patients with idiopathic myopathies (IIM).
METHODS: An international multidisciplinary consensus effort to standardize the conduct and reporting of the myositis clinical trials has been established. Two tools, known as the myositis intention to treat index (MITAX) and the myositis disease activity assessment visual analogue scale (MYOACT), have been developed to capture activity in patients with IIM. In addition, the myositis damage index (MDI) has been devised to assess the extent and severity of damage developing in different organs and systems. These measures have been reviewed by the myositis experts participating in the International Myositis Assessment and Clinical Studies (IMACS) group and have been found to have good face validity and to be comprehensive. The instruments were assessed in two real patient exercises involving patients with adult dermatomyositis and inclusion body myositis.
RESULTS: The reliability of MITAX, MYOACT and MDI, measured by the intraclass correlation coefficient among the physicians, and the inter-rater reliability, as assessed by variation in the physicians' rating of patients, was fair to good for most aspects of the tools. Reliability and inter-rater agreement improved at the second exercise after the participants had completed additional training.
CONCLUSIONS: The MITAX, MYOACT and MDI tools, which are now undergoing validity testing, should enhance the consistency, comprehensiveness and reliability of disease activity and damage assessment in patients with myositis.


Curr Opin Rheumatol. 2003 Nov;15(6):679-90.

Physical activity and disablement in the idiopathic inflammatory myopathies.

Harris-Love MO.

Rehabilitation Medicine Department, National Institute of Health, Bethesda, MD 20892, USA. mlove@nih.gov

PURPOSE OF REVIEW: The sequelae associated with idiopathic inflammatory myopathy (IIM) often result in disability and decreased quality of life. Our understanding of how exercise mitigates disability may be facilitated through the use of a conceptual model. This review describes the enablement-disablement model applied to myositis and explores the role of physical activity in the enablement process. RECENT FINDINGS: National and international organizations have revised their disablement models by acknowledging disability as a relational concept, refining the relationship of disability to quality of life, and incorporating the role of intervention through the enablement process. Disability associated with IIM may be complicated by aging-related comorbidities and decreased physical activity. However, data indicate that both short-term and long-term aerobic training results in improved aerobic capacity and decreased disability in adults with IIM. Strengthening regimens have also resulted in decreased functional limitations and disability for adults with polymyositis and dermatomyositis. While comprehensive exercise programs have not been shown to exacerbate disease activity or damage in people with IIM, their effectiveness for individuals with inclusion body myositis (IBM) remains uncertain.
SUMMARY: Physical activity constitutes a valuable enablement strategy that can reduce disability in adults with IIM. Use of the disablement-enablement model and ICF taxonomy in conjunction with outcomes across disablement domains may augment further investigation of the effectiveness of exercise interventions. Additional research is needed to better understand the relationship between disease severity and optimal exercise dosage, the effects of long-term exercise in children with IIM, and the physiologic response to exercise in people with IBM.


Clin Rehabil. 2003 Feb;17(1):83-7.

The effect of physical exercise following acute disease exacerbation in patients with dermato/polymyositis.

Varju C, Petho E, Kutas R, Czirjak L.

Department of Physical Medicine and Rehabilitation, General Hospital of Szigetvar, Szigetvar, Hungary.

OBJECTIVE: To study the effect of physical exercise shortly after an acute episode of dermato/polymyositis (DM/PM).
DESIGN: Pilot study of a descriptive nature.
SETTING: Rehabilitation unit of a large general hospital.
SUBJECTS: Ten patients 2-3 weeks after an acute phase of DM/PM (early recovery group) and 11 patients in the inactive stage of DM/PM for at least three months (chronic stage group). INTERVENTIONS: Isotonic muscle training consisted of several series of different repeated movements at 65-70% of individual maximal repetition limit. Special training was applied for the respiratory muscles. Relaxing baths, mud packs and massages were also applied.
OUTCOME MEASURES: Dynamometer and spirometer were used for measuring the changes in muscle strength and respiratory function. Disability tests were done before and after therapy.
RESULTS: No disease relapses or decreases in muscle function were seen. In the early recovery group, the average muscle strength improvement was 17 +/- 31 % (p > 0.05) in the proximal muscles and 37 +/- 23% (p < 0.05) in the distal muscles, while the vital capacity also increased by 17 +/- 21% (p < 0.05). In the chronic stage group the average improvement in muscle strength was 46 +/- 34% (p < 0.05) in the proximal muscles and 37 +/- 29% (p < 0.05) in the distal muscles. By the end of the therapy both groups showed improvements in disability tests (p < 0.05).
CONCLUSIONS: Physical training started 2-3 weeks following an acute exacerbation of the disease seems to be useful and safe. Some improvement in muscle strength and respiratory function can be obtained, muscle atrophy due to inactivity may be partially prevented and the level of disability can be decreased.


J Rehabil Med. 2003 Jan;35(1):31-5.

Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction.

Arnardottir S, Alexanderson H, Lundberg IE, Borg K.

Department of Clinical Neuroscience Division of Neurology, Karolinska Hospital, SE-171 76 Stockholm, Sweden. snjolaug.arnardottir@ks.se

OBJECTIVE: To evaluate the safety and effect of a home training program on muscle function in 7 patients with sporadic inclusion body myositis. DESIGN: The patients performed exercise 5 days a week over a 12-week period.
METHODS: Safety was assessed by clinical examination, repeated muscle biopsies and serum levels of creatine kinase. Muscle strength was evaluated by clinical examination, dynamic dynamometer and by a functional index in myositis.
RESULTS: Strength was not significantly improved after the exercise, however none of the patients deteriorated concerning muscle function. The histopathology was unchanged and there were no signs of increased muscle inflammation or of expression of cytokines and adhesion molecules in the muscle biopsies. Creatine kinase levels were unchanged. A significant decrease was found in the areas that were positively stained for EN-4 (a marker for endothelial cells) in the muscle biopsies after training.
CONCLUSION: The home exercise program was considered as not harmful to the muscles regarding muscle inflammation and function. Exercise may prevent loss of muscle strength due to disease and/or inactivity.


Curr Rheumatol Rep. 2001 Aug;3(4):317-24.

The benefits and limitations of a physical training program in patients with inflammatory myositis.

Lawson Mahowald M.

Minneapolis VA Medical Center, Rheumatology Office (111R), One Veterans Drive, Minneapolis, MN 55417, USA. Mahow001@umn.edu

The clinical features of inflammatory myositis are determined by the severity and extent of muscle weakness and systemic manifestations. The benefits and limitations of physical training programs and rehabilitation strategies depend on the clinical phase of the disease and analysis of underlying impairments responsible for functional limitations in the patient. Patients with early stage disease and severe weakness will be treated differently than patients who have responded to medication and are improving. Not all patients will respond to medications; their therapy programs will have different requirements. This article reviews available data on the physiologic responses to exercise in patients with inflammatory muscle diseases. New data support more aggressive approaches to progressive strengthening exercises for patients with inflammatory myositis.


Muscle Nerve. 1997 Oct;20(10):1242-8.

Safety and efficacy of strength training in patients with sporadic inclusion body myositis.

Spector SA, Lemmer JT, Koffman BM, Fleisher TA, Feuerstein IM, Hurley BF, Dalakas MC.

Neuromuscular Diseases Section, NINDS, National Institutes of Health, Bethesda, MD 20892, USA.

We studied the effects of a 12-week progressive resistance strength training program in weakened muscles of 5 patients with sporadic inclusion body myositis (IBM). Strength was evaluated with Medical Research Council (MRC) scale ratings and quantitative isometric and dynamic tests. Changes in serum creatine kinase (CK), lymphocyte subpopulations, muscle size (determined by magnetic resonance imaging), and histology in repeated muscle biopsies were examined before and after training. After 12 weeks, the values of repetition maximum improved in the least weakened muscles, 25-120% from baseline. This dynamic effect was not captured by MRC or isometric muscle strength measurements. Serum CK, B cells, T-cell subsets, and NK cells remained unchanged. Repeat muscle biopsies did not reveal changes in the number and degree of degenerating fibers or inflammation. The size of the trained muscles did not change. We conclude that a supervised progressive resistance training program in IBM patients can lead to gains in dynamic strength of the least weak muscles without causing muscle fatigue and muscle injury or serological, histological, and immunological abnormalities. Even though the functional significance of these gains is unclear, this treatment modality is a safe and perhaps overlooked means of rehabilitation of IBM patients.

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