Several other measures have been developed and partially validated to assess disease activity and damage in certain target organs, such as the skin or muscle, or in certain populations, such as in patients with juvenile dermatomyositis. The tools listed below, including 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]
Cutaneous Assessment Tool (CAT)
Clinical care and therapeutic trials in idiopathic inflammatory myopathies (IIM) require accurate and consistent assessment of cutaneous involvement. The Cutaneous Assessment Tool (CAT) was designed to measure skin activity and damage in IIM. The CAT includes 10 activity, 4 damage and 7 combined lesions. The CAT has been preliminarily validated in a large population of juvenile myositis patients and shown good reliability. The CAT is a promising, semi-quantitative tool to comprehensively assess skin disease activity and damage in IIM.
- Cutaneous Assessment Tool -- Word Format(148KB) Cutaneous Assessment Tool -- PDF Format(274KB)
- Abbreviated Cutaneous Assessment Tool, Binary Version - PDF Format(41KB)
- Huber AM, Lachenbruch PA, Dugan EM, Miller FW, Rider LG; For the Juvenile Dermatomyositis Disease Activity Collaborative Study GroupAdditional members of the Juvenile Dermatomyositis Disease Activity Collaborative Study Group are listed in Appendix A. Alternative scoring of the cutaneous assessment tool in juvenile dermatomyositis: Results using abbreviated formats. Arthritis Rheum. 2008 Mar 15;59(3):352-356. [Pub Med]
- Huber AM, Dugan EM, Lachenbruch PA, Feldman BM, Perez MD, Zemel LS, Lindsley CB, Rennebohm RM, Wallace CA, Passo MH, Reed AM, Bowyer SL, Ballinger SH, Miller FW, Rider LG; Juvenile Dermatomyositis Disease Activity Collaborative Study Group Preliminary validation and clinical meaning of the Cutaneous Assessment Tool in juvenile dermatomyositis. Arthritis Rheum.2008 Feb 15;59(2):214-21. [Pub Med]
- Huber AM, Dugan EM, Lachenbruch PA, Feldman BM, Perez MD, Zemel LS, Lindsley CB, Rennebohm RM, Wallace CA, Passo MH, Reed AM, Bowyer SL, Ballinger SH, Miller FW, Rider LG; Juvenile Dermatomyositis Disease Activity Collaborative Study Group. The Cutaneous Assessment Tool (CAT): development and reliability in juvenile idiopathic inflammatory myopathy. Rheumatology (Oxford). 2007 Oct;46(10):1606-11. [Abstract]
- Dugan EM, Huber AM, Miller FW, and Rider LG; Photoessay of the idiopathic inflammatory myopathies. Dermatology Online Journal. 2009;15(2):1. [Dermatology Online Journal]
Disease Activity Score
This partially validated tool has been developed by Dr. Lauren Pachman's group for assessment of global disease activity in juvenile dermatomyositis. The tool assesses muscle and cutaneous manifestations, including vasculopathic features, based on a bedside clinical assessment. Although the Disease Activity Score is not currently an IMACS Core Set Measure of Disease Activity, IMACS members have agreed to include this measure in future studies of adult and juvenile myositis. The version of the tool posted here captures additional data that is not currently being scored, but is undergoing further testing. This tool will be part of the IMACS Trials Outcomes Data Repository.
- Smith RL, Sundberg J, Shamiyah E, Dyer A, Pachman LM, Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops, J Rheumatol., 2004 Aug, 31(8):1644-9 [Pub Med]
- Bode RK, Klein-Gitelman MS, Miller ML, Lechman TS, Pachman LM, Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence, Arthritis Rheum., 2003 Feb 15, 49(1):7-15 [Pub Med (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12579588)
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI)
The CDASI, developed by Dr. Victoria Werth and colleagues, is a clinician- scored single page instrument that separately measures activity and damage in the skin of dermatomyositis patients for use in clinical practice or clinical/therapeutic studies. The modified CDASI (version 2) is the one in current use. The modified CDASI has 3 activity measures (erythema, scale, and erosion/ulceration) and 2 damage measures (poikiloderma and calcinosis) which are assessed over 15 body areas. In addition, Gottron’s papules on the hands are evaluated both for activity and damage. Lastly, the activity of periungual changes and alopecia is assessed.
- The "Cutaneous Dermatomyositis Area and Severity Index (CDASI)" is copyrighted by the University of Pennsylvania. For academic use, please contact Dr. Werth for permission ( email@example.com ). For commercial use, please contact Dr. Erli Chen for permission ( firstname.lastname@example.org ).
- CDASI Training Tool(1MB)
- Anyanwu CO, Fiorentino D, Chung L, Dzuong C, Wang Y, Okawa J, Carr K, Propert KJ, Werth VP. Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI): characterizing disease severity and assessing responsiveness to clinical change. Br J Dermatol. 2015 May 21. doi: 10.1111/bjd.13915. [Epub ahead of print] [Abstract]
- Goreshi R, Okawa J, Rose M, Feng R, Lee LA, Hansen CB, Bangert CA, Connolly MK, Davis MD, Callen JP,. Fett NM, Fakharzadeh SS, Clarke JT, Werth VP. Evaluation of reliability, validity, and responsiveness of the CDASI and the CAT-BM. J Invest Dermatol. 2012 Apr;132(4):1117-24. [PubMed]
- Goreshi R, Chock M, Foering K, Feng R, Okawa J, Rose M, Fiorentino D, Werth V. Quality of life in dermatomyositis. J Am Acad Dermatol. 2011 Dec;65(6):1107-16. [PubMed]
- Yassaee M, Fiorentino D, Okawa J, Taylor L, Coley C, Troxel AB, Werth VP. Modification of the cutaneous dermatomyositis disease area and severity index, an outcome instrument. Br J Dermatol. 2010 Mar;162(3):669-73. [PubMed]
- Klein RQ, Bangert CA, Costner M, Connolly MK, Tanikawa A, Okawa J, Rose M, Fakharzadeh SS, Fiorentino D, Lee LA, Sontheimer RD, Taylor L, Troxel AB, Werth VP. Comparison of the reliability and validity of outcome instruments for cutaneous dermatomyositis. Br J Dermatol. 2008 Sep;159(4):887-94. [PubMed]
The Functional Index 2 (FI-2)
The FI-2 is a functional outcome developed for patients with adult polymyositis or dermatomyositis assessing muscle endurance in seven muscle groups. Each muscle group is scored as the number of correctly performed repetitions with 60 or 120 maximal number of repetitions depending on muscle group. The FI-2 is a further development of the original Functional Index (FI) and has been validated as to content and construct validity and intra- and inter-rater reliability. The FI-2 can be performed on both right and left sides requiring a maximum of 33 minutes, or just on the dominant side which takes 21 minutes.
Functional Index-2: Training Video
- Alexanderson H, Broman L, Tollbäck 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 (Arthritis Care Res), 2006;55:114-22. [Pub Med]
- Josefson A, Romanus E, Carlsson J. A functional index in myositis. J Rheumatol, 1996;23:1380-4. [Pub Med]
- Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc, 1982;14:377-81. [Pub Med]
Semi-open Muscle Biopsy Technique Using a Conchotome
The semi-open muscle biopsy technique or percutaneous conchotome technique was developed in Scandinavia (Henriksson KG, 1979) and has been adopted as a routine biopsy method in many rheumatology and neurology centers, particularly in Northern Europe. It gives a good yield of muscle tissue for routine histopathology, immunohistochemistry staining and for biochemistry analyses as well as for research on muscle tissue (Dorph C et al 2001). The video and accompanying script provide details about the performance of this procedure. This video was kindly contributed by Drs. Ingrid Lundberg and Maryam Dastmalchi of the Karolinska Institute in Stockholm, Sweden.
For questions, please contact them at:
Dr. Ingrid Lundberg M.D., Ph.D., Professor
Dr. Maryam Dastmalchi, M.D., Ph.D., Consultant
Rheumatology Unit, Karolinska University Hospital and Karolinska Institutet Stockholm, Sweden
- Dorph C, Nennesmo I, Lundberg IE. Percutaneous conchotome muscle biopsy. A useful diagnostic and assessment tool J. Rheumatol, 2001;28:1591-9. [Pub Med]
- Henriksson KG. "Semi-open" muscle biopsy technique. A simple outpatient procedure. Acta Neurol Scand, 1979;59:317-23. [ Pub Med]