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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.

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]

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.

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Related Educational Material:

References:

  1. Ruperto N, Ravelli A, Pistorio A, Ferriani V, Calvo I, Ganser G, Brunner J, Dannecker G, Silva CA, Stanevicha V, Cate RT, van Suijlekom-Smit LW, Voygioyka O, Fischbach M, Foeldvari I, Hilario O, Modesto C, Saurenmann RK, Sauvain MJ, Scheibel I, Sommelet D, Tambic-Bukovac L, Barcellona R, Brik R, Ehl S, Jovanovic M, Rovensky J, Bagnasco F, Lovell DJ, Martini A; Paediatric Rheumatology International Trials Organisation (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). The provisional Paediatric Rheumatology International Trials Organisation/American College of Rheumatology/European League Against Rheumatism Disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: a prospective validation study. Arthritis Rheum. 2008 Jan 15;59(1):4-13. doi: 10.1002/art.23248. [Abstract] 
  2. 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]

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:

References:

  1. Ruperto N, Ravelli A, Pistorio A, Ferriani V, Calvo I, Ganser G, Brunner J, Dannecker G, Silva CA, Stanevicha V, Cate RT, van Suijlekom-Smit LW, Voygioyka O, Fischbach M, Foeldvari I, Hilario O, Modesto C, Saurenmann RK, Sauvain MJ, Scheibel I, Sommelet D, Tambic-Bukovac L, Barcellona R, Brik R, Ehl S, Jovanovic M, Rovensky J, Bagnasco F, Lovell DJ, Martini A; Paediatric Rheumatology International Trials Organisation (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). The provisional Paediatric Rheumatology International Trials Organisation/American College of Rheumatology/European League Against Rheumatism Disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: a prospective validation study. Arthritis Rheum. 2008 Jan 15;59(1):4-13. doi: 10.1002/art.23248. [Abstract] 
  2. 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]

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 similarly to a total of 24 muscle groups, and is also proposed for use for research studies.

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Related Educational Material:

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.

 

MMT Videos: 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

 

MMT Videos: 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]
  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]
  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. [Abstract]

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:

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.

Related Educational Material:

HAQ References:

  1. Ponyi A, Borgulya G, Constantin T, Váncsa A, Gergely L, Dankó K. Functional outcome and quality of life in adult patients with idiopathic inflammatory myositis. Rheumatology (Oxford). 2005 Jan;44(1):83-8. [Abstract] 
  2. 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]

CHAQ References:

  1. Ruperto N, Ravelli A, Pistorio A, Ferriani V, Calvo I, Ganser G, Brunner J, Dannecker G, Silva CA, Stanevicha V, Cate RT, van Suijlekom-Smit LW, Voygioyka O, Fischbach M, Foeldvari I, Hilario O, Modesto C, Saurenmann RK, Sauvain MJ, Scheibel I, Sommelet D, Tambic-Bukovac L, Barcellona R, Brik R, Ehl S, Jovanovic M, Rovensky J, Bagnasco F, Lovell DJ, Martini A; Paediatric Rheumatology International Trials Organisation (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). The provisional Paediatric Rheumatology International Trials Organisation/American College of Rheumatology/European League Against Rheumatism Disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: a prospective validation study. Arthritis Rheum. 2008 Jan 15;59(1):4-13. doi: 10.1002/art.23248. [Abstract] 
  2. 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]
  3. 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]

CMAS References:

  1. Huber AM, Lovell DJ, Pilkington CA, Rennebohm RM, Rider LG. Confusion concerning multiple versions of the childhood myositis assessment scale. Arthritis Care Res (Hoboken). 2014 Apr;66(4):648. doi: 10.1002/acr.22239. [Abstract] 
  2. Quiñones R, Morgan GA, Amoruso M, Field R, Huang CC, Pachman LM. Lack of achievement of a full score on the childhood myositis assessment scale by healthy four-year-olds and those recovering from juvenile dermatomyositis. Arthritis Care Res (Hoboken). 2013 Oct;65(10):1697-701. [Abstract] 
  3. Ruperto N, Ravelli A, Pistorio A, Ferriani V, Calvo I, Ganser G, Brunner J, Dannecker G, Silva CA, Stanevicha V, Cate RT, van Suijlekom-Smit LW, Voygioyka O, Fischbach M, Foeldvari I, Hilario O, Modesto C, Saurenmann RK, Sauvain MJ, Scheibel I, Sommelet D, Tambic-Bukovac L, Barcellona R, Brik R, Ehl S, Jovanovic M, Rovensky J, Bagnasco F, Lovell DJ, Martini A; Paediatric Rheumatology International Trials Organisation (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). The provisional Paediatric Rheumatology International Trials Organisation/American College of Rheumatology/European League Against Rheumatism Disease activity core set for the evaluation of response to therapy in juvenile dermatomyositis: a prospective validation study. Arthritis Rheum. 2008 Jan 15;59(1):4-13. doi: 10.1002/art.23248. [Abstract]
  4. Rennebohm RM, Jones K, Huber AM, Ballinger SH, Bowyer SL, Feldman BM, Hicks J, Katona IM, Lindsley CB, Miller FW, Passo MH, Perez MD, Reed AM, Wallace CA, White PH, Zemel LS, Lachenbruch PA, Hayes JR, Rider LG; Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Normal scores for nine maneuvers of the Childhood Myositis Assessment Scale. Arthritis Rheum. 2004 Jun 15;51(3):365-70. [Abstract] 
  5. 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]
  6. 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]

Laboratory - Muscle Enzymes

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.

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References:

  1. Volochayev R, Csako G, Wesley R, Rider LG, Miller FW. Laboratory Test Abnormalities are Common in Polymyositis and Dermatomyositis and Differ Among Clinical and Demographic Groups. Open Rheumatol J. 2012;6:54-63. doi: 10.2174/1874312901206010054. [Abstract]
  2. 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]
  3. 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]
  4. 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]

Extramuscular Assessment - Myositis Disease Activity Assessment Tool

Description:

This 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).

The Myositis Disease Activity Assessment Tool (MDAAT) has 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.

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References:

  1. Sanner H, Sjaastad I, Flatø B. Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool. Rheumatology (Oxford). 2014 Sep;53(9):1578-85. doi: 10.1093/rheumatology/keu146. [Abstract] 
  2. 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]
  3. 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]
  4. 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).
  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:

References:

  1. 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]