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Disease Activity Core Set Measures

Disease activity assesses the manifestations of myositis which are thought to be reversible that result directly from the inflammatory process. IMACS has developed a consensus on a set of Core Set Domains and Measures for the assessment of disease activity. IMACS recommends these core set measures be included in all myositis therapeutic trials and clinical studies that assess disease activity in patients with myositis. The core set myositis disease activity measures are provided below, with instructions and educational resources for many of the tools.

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The tools are available in the following media types:
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Physician Global Activity - Visual Analogue Scale/Likert

  • Description:

    This partially validated tool measures the global evaluation by the treating physician of the overall disease activity of the patient at the time of assessment using a 10 cm. visual analogue scale and a 5 point Likert scale.

  • Download:

  • Related educational material:

  • Reference:

    1. Rider LG, Feldman BM, Perez MD, Rennebohm RM, Lindsley CB, Zemel LS, Wallace CA, Ballinger SH, Bowyer SL, Reed AM, Passo MH, Katona IM, Miller FW, Lachenbruch PA. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: I. Physician, parent, and patient global assessments. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum. 1997 Nov;40(11):1976-83. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9365086) Exit NIEHS

Patient/Parent Global Activity - Visual Analogue Scale/Likert

  • Description:

    This partially validated tool measures the global evaluation by the patient, or by the parent if the patient is a minor, of the patient's overall disease activity at the time of assessment using a 10 cm. visual analogue scale.

  • Download

  • Reference:

    1. Rider LG, Feldman BM, Perez MD, Rennebohm RM, Lindsley CB, Zemel LS, Wallace CA, Ballinger SH, Bowyer SL, Reed AM, Passo MH, Katona IM, Miller FW, Lachenbruch PA. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: I. Physician, parent, and patient global assessments. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum. 1997 Nov;40(11):1976-83. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9365086) Exit NIEHS

Muscle Strength Testing - Manual Muscle Testing (MMT)

  • Description:

    This partially validated tool assesses muscle strength using manual muscle testing (MMT). A 0 - 10 point scale is proposed for use. This tool is available to IMACS members only.

  • Download:

    • Manual Muscle Testing (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/mmt.pdf)  Download Adobe Reader (29.3 KB)

  • Related educational material:

  • References:

    1. Rider LG, Giannini EH, Harris-Love M, Joe G, Isenberg D, Pilkington C, Lachenbruch PA, Miller FW; International Myositis Assessment and Clinical Studies Group. Defining Clinical Improvement in Adult and Juvenile Myositis. J Rheumatol. 2003 Mar;30(3):603-17. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12610824) Exit NIEHS
    2. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, Zemel LS, Wallace CA, Ballinger SH, Passo MH, Reed AM, Summers RM, White PH, Katona IM, Miller FW, Lachenbruch PA, Rider LG; Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2004 May;50(5):1595-603. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15146430) Exit NIEHS

Functional Assessment Tools - HAQ, CHAQ, CMAS

  • Description:

    These are three partially validated tools to assess physical function. The Health Assessment Questionnaire (HAQ) Disability Index is to be used for adults and the Childhood HAQ (CHAQ) in children. The Childhood Myositis Assessment Score (CMAS) has been validated in children with myositis. IMACS recommends the use of at least one of these tools to assess physical function as a core set measure in therapeutic trials and clinical studies reporting on myositis disease activity.

  • Download

    • HAQ -- Word Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/haq.doc)  Download Microsoft Word Viewer (50.0 KB) HAQ -- PDF Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/haq.pdf)  Download Adobe Reader (89.2 KB)
    • HAQ Instructions (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/05haqinstruct.pdf)  Download Adobe Reader (100 KB) - The IMACS Clinical Trial/Study Outcomes Data Repository uses the scoring of the HAQ Disability Index of the CLINHAQ
    • CHAQ -- Word Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/chaq.doc)  Download Microsoft Word Viewer (49.5 KB) CHAQ -- PDF Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/chaq.pdf)  Download Adobe Reader (108 KB)
    • CHAQ Instructions (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/chaq_instruct.pdf)  Download Adobe Reader (138 KB)
    • CMAS (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/cmas.pdf)  Download Adobe Reader (33.6 KB)
    • CMAS Instructions (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/cmas_instruct.pdf)  Download Adobe Reader (34.5 KB)

  • Related educational material:

    • This is a videotape demonstration of the Childhood Myositis Assessment Scale (CMAS) and its scoring, created by Dr. Robert Rennebohm, Pediatric Rheumatology, Columbus Children's Hospital, Columbus, OH.
      CMASvideodoc.pdf (http://www.niehs.nih.gov/research/resources/collab/imacs/restrict/video/CMASvideodoc.pdf)  Download Adobe Reader (25.7 KB)
      CMAS Video: movie
      Part 1: Demonstration of the CMAS and It's Scoring (http://www.niehs.nih.gov/research/resources/collab/imacs/restrict/video/CMAS.mov)  Download QuickTime
      Part 2: CMAS exam of Three Patients with Juvenile Dermatomyositis - Coming Soon

  • HAQ Reference:

    1. Alexanderson H, Lundberg IE, Stenstrom CH. Development of the myositis activities profile--validity and reliability of a self-administered questionnaire to assess activity limitations in patients with polymyositis/dermatomyositis. J Rheumatol. 2002 Nov;29(11):2386-92. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12415597) Exit NIEHS

  • CHAQ References:

    1. Huber AM, Hicks JE, Lachenbruch PA, Perez MD, Zemel LS, Rennebohm RM, Wallace CA, Lindsley CB, Passo MH, Ballinger SH, Bowyer SL, Reed AM, White PH, Katona IM, Miller FW, Rider LG, Feldman BM; Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Validation of the Childhood Health Assessment Questionnaire in the juvenile idiopathic myopathies. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. J Rheumatol. 2001 May;28(5):1106-11. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11361197) Exit NIEHS
    2. Feldman BM, Ayling-Campos A, Luy L, Stevens D, Silverman ED, Laxer RM. Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire. J Rheumatol. 1995 Feb;22(2):326-31. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7738957) Exit NIEHS

  • CMAS References:

    1. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, Zemel LS, Wallace CA, Ballinger SH, Passo MH, Reed AM, Summers RM, White PH, Katona IM, Miller FW, Lachenbruch PA, Rider LG; Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2004 May;50(5):1595-603. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15146430) Exit NIEHS
    2. Lovell DJ, Lindsley CB, Rennebohm RM, Ballinger SH, Bowyer SL, Giannini EH, Hicks JE, Levinson JE, Mier R, Pachman LM, Passo MH, Perez MD, Reed AM, Schikler KN, Smith M, Zemel LS, Rider LG. Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum. 1999 Oct;42(10):2213-9. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10524696) Exit NIEHS

Laboratory - Muscle Enymes

  • Description:

    This partially validated tool measures the serum activities of at least 2 of the 4 muscle-associated enzymes including creatine phosphokinase (CK), the transaminases (ALT, AST), lactate dehydrogenase (LD) and aldolase.

  • Download

  • References:

    1. Rider LG, Giannini EH, Harris-Love M, Joe G, Isenberg D, Pilkington C, Lachenbruch PA, Miller FW; International Myositis Assesment and Clinical Studies Group. Defining Clinical Improvement in Adult and Juvenile Myositis. J Rheumatol. 2003 Mar;30(3):603-17. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12610824) Exit NIEHS
    2. Ruperto N, Ravelli A, Murray KJ, Lovell DJ, Andersson-Gare B, Feldman BM, Garay S, Kuis W, Machado C, Pachman L, Prieur AM, Rider LG, Silverman E, Tsitsami E, Woo P, Giannini EH, Martini A; Paediatric Rheumatology International Trials Organization (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis. Rheumatology (Oxford). 2003 Dec;42(12):1452-9. Epub 2003 Jun 27. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12832713) Exit NIEHS
    3. Miller FW, Rider LG, Chung YL, Cooper R, Danko K, Farewell V, Lundberg I, Morrison C, Oakley L, Oakley I, Pilkington C, Vencovsky J, Vincent K, Scott DL, Isenberg DA; International Myositis Outcome Assessment Collaborative Study Group. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology (Oxford). 2001 Nov;40(11):1262-73. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11709610) Exit NIEHS

Extramuscular Assessment - Myositis Disease Activity Assessment Tool

  • Description:

       This partially validated tool measures the degree of disease activity of extra-muscular organ systems and muscle. This is a combined tool that includes the MYOSITIS DISEASE ACTIVITY ASSESSMENT VISUAL ANALOGUE SCALES (MYOACT), which is a series of physician's assessments of disease activity of various organ systems modified from the Vasculitis Activity Index (QE Whiting-O'Keefe et al. 1999, Arthritis and Rheumatism 42: 2365-71), and the MYOSITIS INTENTION TO TREAT ACTIVITY INDEX (MITAX), which is modified from the BILAG approach to assess disease activity in lupus (EM Hay et al., 1993, Q J Medicine, 86: 447-58). The MITAX is composed of a series of organ-specific questions relating to the presence or absence of the clinical feature and the degree of treatment needed for it (intention to treat).

    Two versions of the tool are currently available, and the 0-4 version is recommended for use in future myositis clinical studies. The 0 - 4 version will be available in the IMACS Outcomes Repository.

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  • Download



    • Myositis Disease Activity Assessment Tool, A - E, version 2 (2005) -- Word Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/mdaat_2005_a-e_ver2.doc)  Download Microsoft Word Viewer (188 KB) Myositis Disease Activity Assessment Tool, A - E, version 2 (2005) -- PDF Format (http://www.niehs.nih.gov/research/resources/collab/imacs/docs/activity/mdaat_2005_a-e_ver2.pdf)  Download Adobe Reader (241 KB)

      This instrument was modified from the original version to retrospectively validate the MITAX. The tool scores the MITAX portion using an A - E scale directly, based on the degree of disease activity of each clinical feature and the degree of treatment needed for that feature. The tool has been partially validated in adult myositis clinical trials from NIH and in juvenile myositis natural history studies for content and construct validity, sensitivity to change, and inter-rater reliability (references and unpublished data). This version has primarily been used for retrospective validation of the tool and is not recommended for prospective studies.

  • References:

    1. 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). 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. Rheumatology (Oxford). 2004 Jan;43(1):49-54. Epub 2003 Jul 16. Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12867580) Exit NIEHS
    2. Pilkington C, K Murray, D Isenberg, V Farewell, J Davidson, B Feldman, A Ravelli, R Rennebohm, C Ryder, E Allen, F Miller, L Rider. Development of disease activity and damage indices for myositis: Initial testing of four tools in juvenile dermatomyositis. Arthritis Rheum. 2001 Sept.; 44 (suppl.): S294 (abstract).
    3. Rider L, A Schiffenbauer, M Villalba, E Adams, G Joe, M Harris-Love, L James-Newton, J Hicks, C Pilkington, D Isenberg, P Lachenbruch, F Miller, and the JDM Disease Activity Collaborative Study Group. Extra muscular disease activity is frequent in adult and juvenile idiopathic inflammatory myopathies (IIM) and does not correlate with other myositis activity measures. Arthritis Rheumatism. 2002 Sept.; 46 (suppl.): S612 - 613 (abstract).
    4. Sultan SM, Allen E, Cooper R, Kiely P, Oddis, C, Lundberg I, Vencovsky J, Isenberg DA. Inter-rater reliability of two disease activity and damage tools in patients with idiopathic inflammatory myopathy- repot of 105 patients. Arthritis and Rheumatism. 2004 Sept; 40 (9 suppl.): S668 (abstract).

  • Related educational material:

USA.gov Department of Health & Human Services National Institutes of Health
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Last Reviewed: October 30, 2007