|Ahead of print publication
Patient-centred outcomes for monitoring disease remotely in idiopathic inflammatory myopathies
Arpit Mago1, R Naveen2, Johannes Knitza3, Samuel Katsuyuki Shinjo4, Latika Gupta5, Rohit Aggarwal6
1 Department of Clinical Medicine, Jawaharlal Nehru Medical College, Belgaum, Karnataka, India
2 Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
3 Department of Internal Medicine 3, Friedrich-Alexander Universität Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany
4 Division of Rheumatology, Hospital das Clínicas, Faculty of Medicine, University of São Paulo, São Paulo, Brazil
5 Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India; Department of Rheumatology, Royal Wolverhampton Hospitals NHSTrust, Wolverhampton, WV10 0QP, United Kingdom; Division of Musculoskeletal and Dermatological Sciences, Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester; Sandwell and West Birmingham Hospitals NHSTrust, Birmingham, UK
6 Department of Medicine, University of Pittsburgh, Pittsburgh, USA
|Date of Submission||04-Aug-2021|
|Date of Acceptance||30-Jan-2022|
|Date of Web Publication||05-Apr-2022|
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this URL:|
Mago A, Naveen R, Knitza J, Shinjo SK, Gupta L, Aggarwal R. Patient-centred outcomes for monitoring disease remotely in idiopathic inflammatory myopathies. Indian J Rheumatol [Epub ahead of print] [cited 2022 Oct 1]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=342531
Idiopathic inflammatory myopathies (IIMs) are heterogeneous, systemic autoimmune diseases with varied organ system involvement, which cause significant morbidity and mortality. Their multisystem nature calls for a detailed evaluation of various organ systems, including the muscle, joints, skin, and lungs. The global COVID-19 pandemic has accelerated the shift of in-person clinical practice to virtual consulting necessitating a wider use of patient-centred outcomes (PCOMs) and device-based objective measures of disease assessment.,, In such times, the evaluation of complex rheumatic diseases (RDs) such as IIMs may be a particularly daunting task.
Currently validated myositis core set measures; Manual Muscle Testing (MMT), Myositis Disease Activity Assessment Tool (MDAAT), Physician global disease activity (MD global), Disease Activity Score (DAS), are highly dependent on the physician as well as require in-person visits with examination time ranging from 15 min to 60 min in combined muscle groups, impacting the number of patients that can be evaluated by a physician. This also leads to significant limitations regarding the remote evaluation of patients through teleconsultation.
MMT has been used widely for muscle strength assessment because of its ease of use, reproducibility of results across varied clinical settings, and assessment of muscle weakness from no strength to full strength. It tests the full range of 24 muscle groups against gravity, and abbreviated versions are now available to evaluate 8 or 9 groups of muscles in the MMT-8 and MMT-9, respectively. For better sensitivity, it has also been expanded to include a scale ranging from 0 to 10. However, there are several disadvantages, which impede its usage including:
- Prolonged time consumption
- Patient fatigue
- Occasional muscle pain that making muscle testing unpleasant and stressful
- Lack of co-operation from younger patients
- Continuous muscle testing in different positions causes muscle fatigue altering the reliability of results.
In addition, the existing monitoring using MMT involves only muscle strength assessment, but strength and endurance are two distinct components of muscle function that need to be recorded to improve patient outcomes. This helps in overcoming the ceiling effects of the widely used MMT, hence the version 5.0 incorporates muscle endurance as an outcome measure through validation against a core set of assessments and standard measures.
With the ongoing pandemic that has ushered in an era of digital transformation in health care, several tools are being explored for remote disease monitoring, a realm that may assume larger importance for optimal disease management in future., Several PCOMs typically used for the assessment of patients with IIMs include the Patient Global Assessment of Disease Activity and the Health Assessment Questionnaire – Disability Index as endorsed by International Myositis Assessment and Clinical Studies Group, and the Myositis Activity Profile. In addition, several generic PCOMs have also been explored in patients with IIMs. These include the Medical Outcomes Study Short Form-36, the Nottingham Health Profile, the Arthritis Impact Measurement Scale-2, and the Patient-Reported Outcomes Measurement Information System Measures). Recently, more specific muscle examination maneuvers have been tested to gain insight into the presence or absence of muscle weakness remotely. These include the Arm Lift test, Two-Minute Walk Distance (2MWD), Sit to Stand Test, and Timed Up and Go Test [Table 1].
|Table 1: Device-independent patient-centred outcomes in assessing muscle strength in idiopathic inflammatory myopathies|
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The PCOMs can be classified into two types: Device-dependent tests and device-independent tests. Device-dependent tests include hand-held dynamometry, accelerometer-derived physical activity, and the use of metronome devices for functional testing such as the Function Index-2 [Table 2]. These devices are not always accessible by patients at home, especially in developing countries. Device-independent tests, on the other hand, can be performed easily without any complex devices except for a stopwatch or a timer. They can be done by patients at home remotely, with the ability to transfer the data to their doctors for longitudinal monitoring. The tests can also be divided into time-dependent and time-independent tests. In time-dependent tests, the activity is performed within a stipulated time. The outcome measured is the number of times the person can perform the activity within the given timeframe. In contrast, time-independent tests measure the time taken to do a particular activity once or a fixed number of times. Time-independent tests typically demonstrate a floor effect. In other words, if the patient is not able to perform the task, the time taken for the same might be infinite and would be improper. Therefore, the ideal PCOMs are time-dependent and device-independent tests that are simple, reliable, and repeatable, so these can be better aids for the remote assessment of muscle strength and functionality.
|Table 2: Device-based patient-centred outcomes in assessing muscle strength in idiopathic inflammatory myopathies|
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The development of remote PCOM for the assessment of patients with IIM is marked by several unmet needs. Most importantly, PCOMs are currently incapable of clearly distinguishing active disease from inactive disease. In addition, PCOMs might report muscle weakness due to disease activity, damage, fatigue, or other psychological or environmental factors. Furthermore, some PCOMs are validated, but many are not. There is also a need for PCOMs to cover all domains of disease, including involvement of various organs (skin, lungs, and cardiac apart from the musculoskeletal system), functionality, damage, and quality of life. For example, currently, there is no patient-reported cutaneous assessment tool equivalent to the Cutaneous Dermatomyositis Disease Area and Severity Index score that is entered by a physician. Differences between the self-reporting of several IIM subgroups using each PROM are also an entirely unexplored area. Finally, there is an immediate need to appreciate any differences in the reporting of PROMs when assessed at the clinic and when self-reported by patients from their homes. The latter may require accounting for the interplay between the sociocultural, cognitive, and technological factors while recording these PCOMs. With the advent of better instruments and devices for remote monitoring, these irregularities may be reduced in the years to follow.
Thus, the need of the hour is to devise and study various novel PCOMs for remote assessment. This includes designing simplified measures, testing them in different populations to distinguish active from inactive disease, and further validate responsiveness to change with treatment. Further studying the effect of disease activity and damage on each of these novel PCOMs is the first step in establishing a revised, reliable, and sustainable model of remote health care in complex RDs such as IIM. Herein, based on the authors’ experience, we report a collation of various practical and feasible remote assessment measures that could be potentially useful to assess muscle function remotely [Table 1]. Some of these have been explored in various RDs, whereas others have been pilot tested in single IIM cohorts or are currently under investigation.
As the health-care system increasingly migrates to electronic systems, it is also important to consider how PCOMs can be embedded within these systems for collection, scoring, and data displays to provide information to providers and patients in making shared medical decisions.,,, Digital information systems could facilitate continuous monitoring through the collection of Patient reported Outcomes (PRO) and Health-related quality of life (HRQOL). This could aid in dynamic and real-time practical alterations of treatment strategies and provide references for the frequency of follow-up visits. The future lies in guiding patients with established diagnoses such as IIMs to follow up via teleconsultation follow-ups that include recorded outcome measures, nurse-led care, and tele-triaging. Smartphone apps have evolved during the COVID-19 pandemic to provide clinical guidance using technologies and metrics such as Mobile Application Rating Scale, to ensure credibility and tolerability of the technology assessing the various mobile apps for overall quality, engagement, functionality, esthetics, and information.,
Patient-centred outcome measures, when used alongside clinical measures, add a unique perspective on disease activity as perceived by the patient. PCOMs should be implemented in all patient care to improve the patient-physician relationship and shared decision-making as the values of the patient and physician may not be equivalent. Refinement of IIM-specific instruments is necessary for high-quality care and research.
| References|| |
Gupta L, Chinoy H. Monitoring disease activity and damage in adult and juvenile idiopathic inflammatory myopathy. Curr Opin Rheumatol 2020;32:553-61.
Sanjeevkumar Gaur P, Gupta L. Changing research paradigm in the face of a global pandemic: Foreseeable impact and adaptive measures in academic research in the future. Proc Shevchenko Sci Soc Med Sci [Internet]. 2020;62. Available from: https://mspsss.org.ua/index.php/journal/article/view/31
. [Last accessed on 2022 Mar 25].
Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, et al
. Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res (Hoboken) 2011;63 Suppl 11:S118-57.
Nyberg A, Törnberg A, Wadell K. Correlation between limb muscle endurance, strength, and functional capacity in people with chronic obstructive pulmonary disease. Physiother Can 2016;68:46-53.
Mehta P, Gupta L. Combined case record forms for collaborative datasets of patients and controls of idiopathic inflammatory myopathis. Indian J Rheumatol 2020;15:S191-3.
Saygin D, Oddis CV, Moghadam-Kia S, Rockette-Wagner B, Neiman N, Koontz D, et al
. Hand-held dynamometry for assessment of muscle strength in patients with inflammatory myopathies. Rheumatology (Oxford) 2021;60:2146-56.
Oldroyd A, Little MA, Dixon W, Chinoy H. A review of accelerometer-derived physical activity in the idiopathic inflammatory myopathies. BMC Rheumatol 2019;3:41.
Busija L, Pausenberger E, Haines TP, Haymes S, Buchbinder R, Osborne RH. Adult measures of general health and health-related quality of life: Medical outcomes study short form 36-item (SF-36) and short form 12-Item (SF-12) health surveys, nottingham health profile (NHP), sickness impact profile (SIP), medical outcomes study short form 6D (SF-6D), health utilities index mark 3 (HUI3), quality of well-being scale (QWB), and assessment of quality of life (AQoL). Arthritis Care Res (Hoboken) 2011;63 Suppl 11:S383-412.
Aggarwal R, Marder G, Koontz DC, Nandkumar P, Qi Z, Oddis CV. Efficacy and safety of adrenocorticotropic hormone gel in refractory dermatomyositis and polymyositis. Ann Rheum Dis 2018;77:720-7.
Bingham CO 3rd
, Noonan VK, Auger C, Feldman DE, Ahmed S, Bartlett SJ. Montreal Accord on Patient-Reported Outcomes (PROs) use series – Paper 4: Patient-reported outcomes can inform clinical decision making in chronic care. J Clin Epidemiol 2017;89:136-41.
Mago A, Aggarwal V, Gupta L. Telerheumatology and its interplay with patient-initiated care. Rheumatol Int 2021;41:1883-4.
Krusche M, Klemm P, Grahammer M, Mucke J, Vossen D, Kleyer A, et al
. Acceptance, usage, and barriers of electronic patient-reported outcomes among German rheumatologists: Survey study. JMIR Mhealth Uhealth 2020;8:e18117.
Doeleman M, Roock S, Buijsse N, Klein M, Bonsel G, Seyfert-Margolis V et al
. Monitoring patients with juvenile idiopathic arthritis using health-related quality of life. Pediatr Rheumatol Online J. 2021;19:40.
Kernder A, Morf H, Klemm P, Vossen D, Haase I, Mucke J, et al
. Digital rheumatology in the era of COVID-19: Results of a national patient and physician survey. RMD Open 2021;7:e001548.
Knitza J, Tascilar K, Messner EM, Meyer M, Vossen D, Pulla A, et al
. German mobile apps in rheumatology: Review and analysis using the mobile application rating scale (MARS). JMIR Mhealth Uhealth 2019;7:e14991.
Davalbhakta S, Advani S, Kumar S, Agarwal V, Bhoyar S, Fedirko E, et al
. A systematic review of smartphone applications available for corona virus disease 2019 (COVID19) and the assessment of their quality using the mobile application rating scale (MARS). J Med Syst 2020;44:164.
Kataria S, Ravindran V. Digital health: A new dimension in rheumatology patient care. Rheumatol Int 2018;38:1949-57.
Wolthers TO, Wolthers OD. Telephone consultation as a substitute for face-to-face consultation during the COVID-19 pandemic. Dan Med J 2020;67:A04200300.
Agarwal S, Kiely PD. Two simple, reliable and valid tests of proximal muscle function, and their application to the management of idiopathic inflammatory myositis. Rheumatology (Oxford) 2006;45:874-9.
Alcazar J, Kamper RS, Aagaard P, Haddock B, Prescott E, Ara I, et al
. Relation between leg extension power and 30-s sit-to-stand muscle power in older adults: Validation and translation to functional performance. Sci Rep 2020;10:16337.
Tolk JJ, Janssen RP, Prinsen CS, van der Steen MM, Zeinstra SM, Reijman M. Measurement properties of the OARSI core set of performance-based measures for hip osteoarthritis: A prospective cohort study on reliability, construct validity and responsiveness in 90 hip osteoarthritis patients. Acta Orthop 2019;90:15-20.
Hirose G. Shinkeigaku R. Clin Neurol2015;55:455-8. [doi: 10.5692/clinicalneurol.cn-000693].
Tarentino AL, Maley F. A comparison of the substrate specificities of endo-beta-N-acetylglucosaminidases from Streptomyces griseus
and Diplococcus pneumoniae
. Biochem Biophys Res Commun 1975;67:455-62.
Landon-Cardinal O, Devilliers H, Chavarot N, Mariampillai K, Rigolet A, Hervier B, et al
. Responsiveness to change of 5-point MRC SCALE, endurance and functional evaluation for assessing myositis in daily clinical practice. J Neuromuscul Dis 2019;6:99-107.
Jackson CE, Barohn RJ, Gronseth G, Pandya S, Herbelin L; Muscle Study Group. Inclusion body myositis functional rating scale: A reliable and valid measure of disease severity. Muscle Nerve 2008;37:473-6.
Baschung Pfister P, de Bruin ED, Sterkele I, Maurer B, de Bie RA, Knols RH. Manual muscle testing and hand-held dynamometry in people with inflammatory myopathy: An intra- and interrater reliability and validity study. PLoS One 2018;13:e0194531.
Rockette-Wagner B, Saygin D, Moghadam-Kia S, Oddis C, Landon-Cardinal O, Allenbach Y, et al
. Reliability, validity and responsiveness of physical activity monitors in patients with inflammatory myopathy. Rheumatology (Oxford). 2021;60:5713-23. doi: 10.1093/rheumatology/keab236. PMID: 33714992.
Alexanderson H, Broman L, Tollbäck A, Josefson A, Lundberg IE, Stenström CH. Functional index-2: Validity and reliability of a disease-specific measure of impairment in patients with polymyositis and dermatomyositis. Arthritis Rheum 2006;55:114-22.
[Table 1], [Table 2]