Skip Navigation

Your Environment. Your Health.

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.

 

These and other myositis activity measures, as well as their validation, and performance characteristics, have been reviewed in the following article:

  • Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, Herbelin L, Barohn R, Isenberg D, Miller FW. 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 Nov;63 Suppl 11:S118-57. doi: 10.1002/acr.20532. Review. [Abstract (http://www.ncbi.nlm.nih.gov/pubmed/22588740) ]

 

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:

Physician Global Activity -- Word Format (30KB)

Physician Global Activity -- PDF Format (67KB)

 

Related educational material:

Example Cases for Disease Activity Assessment -- Word Format (138KB)

Example Cases for Disease Activity Assessment -- PDF Format (169KB)

 

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) ]

 

 

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

Patient/Parent Global Activity -- Word Format (30KB)

Patient/Parent Global Activity -- PDF Format (102KB)

 

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) ]

 

 

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. An abbreviated group of 8 proximal, distal, and axial muscles performs similiarly to a total of 24 muscle groups, and is also proposed for use for research studies.

 

Download:

Manual Muscle Testing Scoring Sheet (106KB)

 

Related educational material:

Muscle Grading and Testing Procedures (72KB)

MMT8 Grading and Testing Procedures (for the Abbreviated 8 Muscle Groups) (2MB)

The Manual Muscle Test: Meeting the Challenge of the Therapeutic Trial (402KB)

Myositis Core Set Measures of Activity, including the MMT8 and Preliminary Definitions of Improvement (234KB)

The Manual Muscle Test: Moving Towards Standardization (467KB)

Manual Muscle Testing in the Pediatric Population (66KB)

Manual Muscle Test Considerations for Assessing Strength in Myositis (1MB)

 

MMT Videos:

Manual Muscle Test

 

This is a videotape demonstrating manual muscle testing of a core set of muscles used in the examination of myositis patients.
The videotape was created by Dr. Michael Love, Physical Therapy Section, Department of Rehabilitation Medicine, National Institutes of Health, Bethesda, MD.

 

The Manual Muscle Test: Meeting the Challenge of the Therapeutic Trial

 

This video reviews operational definitions of muscle strength, the utility of the manual muscle test (MMT) and other strength assessment methods, threats to MMT validity and reliability, and MMT grading criteria, focusing on the Kendall Scale.
Michael Harris-Love, D.Sc., MPT

 

The Manual Muscle Test: Moving Towards Standardization

 

This video provides rationale for the MMT-8a and more detailed procedures for testing each of the muscle groups contained in the MMT-8.
Michael Harris-Love, D.Sc., MPT

 

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) ]

     

  2. Harris-Love MO, Shrader JA, Koziol D, Pahlajani N, Jain M, Smith M, Cintas HL, McGarvey CL, James-Newton L, Pokrovnichka A, Moini B, Cabalar I, Lovell DJ, Wesley R, Plotz PH, Miller FW, Hicks JE, Rider LG; Distribution and severity of weakness among patients with polymyositis, dermatomyositis, and juvenile dermatomyositis. Rheumatology (Oxford). 2009 Feb;48(2):134-9. [Pub Med (http://www.ncbi.nlm.nih.gov/pubmed/19074186?itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum&ordinalpos=7) ]

     

  3. Rider LG, Koziol D, Giannini EH, Jain MS, Smith MR, Whitney-Mahoney K, Feldman BM, Wright SJ, Lindsley CB, Pachman LM, Villalba ML, Lovell DJ, Bowyer SL, Plotz PH, Miller FW, Hicks JE; Validation of manual muscle testing and a subset of eight muscles for adult and juvenile idiopathic inflammatory myopathies. Arthritis Care Res (Hoboken). 2010 Apr;62(4):465-72. [Pub Med (http://www.ncbi.nlm.nih.gov/pubmed/20391500) ]

 

 

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 (50KB)

HAQ Instructions (100KB)  

HAQ Instructions (100KB) - The IMACS Clinical Trial/Study Outcomes Data Repository uses the scoring of the HAQ Disability Index of the CLINHAQ

CHAQ -- Word Format (49KB)

CHAQ -- PDF Format (108KB)

CHAQ Instructions (138KB)

CMAS (33KB)

CMAS Instructions (34KB)

 

Related educational material:

CMASvideodoc.pdf (25KB)

 

CMAS Video:

Part 1: Demonstration of the CMAS and It's Scoring

 

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.

 

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) ]

 

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) ]

     

  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) ]

 

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) ]

     

  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) ]

 

 

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

Laboratory - Muscle Enzymes (11KB)

 

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) ]

     

  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) ]

     

  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, Int Myositis Outcome Assessment C. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology. 2001; 40(11):1262-1273. [Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11709610) ]

 

 

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.

 

Download

Myositis Disease Activity Assessment Tool, 0 - 4, version 2 (2005) -- Word Format (111KB)
Myositis Disease Activity Assessment Tool, 0 - 4, version 2 (2005) -- PDF Format (159KB)

Glossary for Myositis Disease Activity Assessment Tool, 0 - 4, version 2 (2005) -- Word Format (53KB)
Glossary for Myositis Disease Activity Assessment Tool, 0 - 4, version 2 (2005) -- PDF Format (109KB)

Scoring of Myositis Disease Activity Assessment Tool, 0 - 4 (2009) - Word Format (68KB)
Scoring of Myositis Disease Activity Assessment Tool, 0 - 4 (2009) - PDF Format (54KB)

 

The Myositis Disease Activity Assessment Tool (MDAAT) is the tool that has been developed by IMACS members and undergone inter-rater reliability testing in adult and juvenile myositis patients in workshops led by Professor David Isenberg and Dr. Clarissa Pilkington. The MITAX is scored on a 0 - 4 scale, based on worsening or improvement in specific clinical features and their correlation with the intention to treat. The tool has undergone a major international reliability and validity exercise in six adult myositis centers, in order to validate the instrument based on the correlation with the intention to treat (references and unpublished data). The version posted here is based on the results of these studies and is the current version recommended for use in prospective trials and clinical studies. This will be the version available in the IMACS Outcomes Repository.

 

References:

  1. Sultan SM, Allen E, Oddis CV, Kiely P, Cooper RG, Lundberg IE, Vencovsky J, Isenberg DA. Reliability and validity of the myositis disease activity assessment tool. Arthritis Rheum. 2008 Nov; 58(11):3593-9. [Pub Med (http://www.ncbi.nlm.nih.gov/pubmed/18975333?ordinalpos=4&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum) ]

     

  2. 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) ]

     

  3. 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).

     

  4. 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).

     

  5. 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:

Teaching Slide Collection of the Cutaneous Manifestations of the Myositis Disease Activity Assessment Tool (1MB)

MDAAT physician global disease activity examples -- Word Format (59KB)

MDAAT physician global disease activity examples -- PDF format (85KB)

MDAAT case examples -- Word Format (50KB)

MDAAT case examples -- PDF format (73KB)

Back to Top