Overview

Over the last decade IMACS has undertaken a number of projects aimed at achieving its goals of improving the lives of children and adults who suffer from myositis by discovering better therapies and understanding the causes of these diseases. Central to these efforts have been a number of studies to develop consensus and standards on the conduct and reporting of adult and juvenile myositis investigations, including outcome measures, clinical trial design and classification of patients with myositis. Listed here is information on the studies that IMACS has now completed, with associated publications.

Completed IMACS Research Projects

IMACS Project I. Developing and Validating Core Set Measures of Myositis Disease Activity and Damage

Primary Objectives: 
Develop and validate new tools to assess disease activity and damage for patients with adult and juvenile dermatomyositis and polymyositis

Start Date: 2000
Completion Date: 2010

Lead Investigators:

Lisa G. Rider, M.D.
Head and Senior Clinician

Tel 301-451-6272
[email protected]

NIH Intramural Profile Lisa G. Rider, M.D.

10 CENTER DR

NIHBC 10 - CRC - HATFIELD

Bethesda, MD 20892-1301

Frederick W. Miller, M.D., Ph.D. (Retired)
Scientist Emeritus

Tel 984-287-3593
[email protected]

NIH Intramural Profile Frederick W. Miller, M.D., Ph.D. (Retired)

111 TW ALEXANDER DR

David P Rall Building

Research Triangle Park, NC 27709

Delivery Instructions

David A. Isenberg, M.D., FRCP

[email protected]

Publications:

1. 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 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.]
2. Huber AM et al. Validation and clinical significance of the childhood myositis assessment scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2004; 50(5):1595-603. [Abstract Huber AM et al. Validation and clinical significance of the childhood myositis assessment scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2004; 50(5):1595-603.]
3. Isenberg DA et al. 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; 43(1): 49-54. [Abstract Isenberg DA et al. 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; 43(1): 49-54.]
4. Rennebohm RM et al. Normal scores for nine maneuvers of the Childhood Myositis Assessment Scale. Arthritis Rheum. 2004; 51(3):365-70. [Abstract Rennebohm RM et al. Normal scores for nine maneuvers of the Childhood Myositis Assessment Scale. Arthritis Rheum. 2004; 51(3):365-70.]
5. 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 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.]
6. Isenberg DA et al. 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; 43(1): 49-54. [Abstract Isenberg DA et al. 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; 43(1): 49-54.]
7. Huber AH et al. Preliminary validation and clinical meaning of the cutaneous assessment tool (CAT) in juvenile dermatomyositis. Arthritis Rheum. 2008; 59:214-221. [Abstract Huber AH et al. Preliminary validation and clinical meaning of the cutaneous assessment tool (CAT) in juvenile dermatomyositis. Arthritis Rheum. 2008; 59:214-221.]
8. Huber AH et al. Alternative scoring of the cutaneous assessment tool (CAT) in juvenile dermatomyositis: Results using abbreviated formats. Arthritis Rheum. 2008; 59:352-356. [Abstract Huber AH et al. Alternative scoring of the cutaneous assessment tool (CAT) in juvenile dermatomyositis: Results using abbreviated formats. Arthritis Rheum. 2008; 59:352-356.]
9. Rider LG et al. Damage extent and predictors in adult and juvenile dermatomyositis and polymyositis using the Myositis Damage Index (MDI). Arthritis Rheum. 2009;60:3425-35. [Abstract Rider LG et al. Damage extent and predictors in adult and juvenile dermatomyositis and polymyositis using the Myositis Damage Index (MDI). Arthritis Rheum. 2009;60:3425-35.]
10. Lachenbruch PA et al. On determining the effects of therapy on disease damage in non-randomized studies with multiple treatments: a study of juvenile myositis. Communications in Statistics – Theory and Methods. 2009;38:3268-81. [Abstract Lachenbruch PA et al. On determining the effects of therapy on disease damage in non-randomized studies with multiple treatments: a study of juvenile myositis. Communications in Statistics – Theory and Methods. 2009;38:3268-81.]
11. Dugan EM, Huber AM, Miller FW, and Rider LG; Photoessay of the idiopathic inflammatory myopathies. Dermatology Online Journal. 2009;15(2):1. [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 Dugan EM, Huber AM, Miller FW, and Rider LG; Photoessay of the idiopathic inflammatory myopathies. Dermatology Online Journal. 2009;15(2):1.]
12. 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 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.]
13. 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 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.]

IMACS Project II. Determining Clinically Meaningful Change in Core Set Activity Measures and Developing Preliminary Definitions of Improvement

IMACS Project III. Consensus Guidelines for the Design and Conduct of Myositis Clinical Trials

Primary Objectives:
Develop consensus in the design of myositis clinical trials, including classification criteria for myositis.

Start Date: 2002
Completion Date: 2005

Lead Investigators:

Chet Oddis

[email protected]

Lisa G. Rider, M.D.
Head and Senior Clinician

Tel 301-451-6272
[email protected]

NIH Intramural Profile Lisa G. Rider, M.D.

10 CENTER DR

NIHBC 10 - CRC - HATFIELD

Bethesda, MD 20892-1301

Frederick W. Miller, M.D., Ph.D. (Retired)
Scientist Emeritus

Tel 984-287-3593
[email protected]

NIH Intramural Profile Frederick W. Miller, M.D., Ph.D. (Retired)

111 TW ALEXANDER DR

David P Rall Building

Research Triangle Park, NC 27709

Delivery Instructions

Ann Reed, M.D.
Professor and Chair, Department of Pediatrics, Physician-in-Chief, Duke Children's Hospital

Tel 919-681-4080
[email protected]

Duke University Medical Center

Box 3352

Durham, NC 27710

Publications:

1. Oddis CV et al. International consensus guidelines for therapeutic trials in the idiopathic inflammatory myopathies. Arthritis Rheum. 2005; 52(9):2607-2615. [Abstract Oddis CV et al. International consensus guidelines for therapeutic trials in the idiopathic inflammatory myopathies. Arthritis Rheum. 2005; 52(9):2607-2615.]

IMACS Project IV. Dyslipidemia in Myositis Survey

Primary Objectives: 
To examine the frequency of lipid profile abnormalities in patients with myositis.

Start Date: 2009
Completion Date: 2010

Lead Investigators:

Christina Charles-Schoeman

[email protected]

Publications:

1. Charles-Schoeman C, Amjadi SS, Paulus HE; International Myositis Assessment and Clinical Studies Group. Treatment of dyslipidemia in idiopathic inflammatory myositis: results of the International Myositis Assessment and Clinical Studies Group survey, Clin Rheumatol. 2012 Aug;31(8):1163-8. [Abstract Charles-Schoeman C, Amjadi SS, Paulus HE; International Myositis Assessment and Clinical Studies Group. Treatment of dyslipidemia in idiopathic inflammatory myositis: results of the International Myositis Assessment and Clinical Studies Group survey, Clin Rheumatol. 2012 Aug;31(8):1163-8.]

IMACS Project V. Identification of a Candidate Core-set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies

Primary Objectives:
To develop consensus on a candidate core-set of fitness and strength tests for children and adults with myositis

Start Date: 2013
Completion Date: 2015

Lead Investigators:

Djamilla K.D. van der Stap

[email protected]

Tim Takken

[email protected]

Publications:

1. van der Stap DK, Rider LG, Alexanderson H, Huber AM, Gualano B, Gordon P, van der Net J, Mathiesen P, Johnson LG, Ernste FC, Feldman BM, Houghton KM, Singh-Grewal D, Kutzbach AG, Munters LA, Takken T; International Myositis Assessment and Clinical Studies Group. Proposal for a Candidate Core Set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies. J Rheumatol. 2016 Jan;43(1):169-76. [Abstract van der Stap DK, Rider LG, Alexanderson H, Huber AM, Gualano B, Gordon P, van der Net J, Mathiesen P, Johnson LG, Ernste FC, Feldman BM, Houghton KM, Singh-Grewal D, Kutzbach AG, Munters LA, Takken T; International Myositis Assessment and Clinical Studies Group. Proposal for a Candidate Core Set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies. J Rheumatol. 2016 Jan;43(1):169-76.]

IMACS Project VI. International Myositis Classification Criteria Project

Primary Objectives: 
To develop new preliminarily validated classification criteria for adult and juvenile dermatomyositis, adult polymyositis and inclusion body myositis.

For further information on the International Myositis Classification Criteria Project, please see Classification Criteria.

Start Date: 2004
Completion Date: Ongoing

Lead Investigators:

Ingrid Lundberg, M.D., Ph.D.

Tel 011-46-8-5177-3080
[email protected]

Rheumatology Unit

Department of Medicine

Karolinska University Hospital

Solna, Karolinska Institutet

SE- 171 76 Stockholm, Sweden

Publications:

1. Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Torre IG, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017 Dec;76(12):1955-1964. doi: 10.1136/annrheumdis-2017-211468. [Abstract Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Torre IG, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017 Dec;76(12):1955-1964. doi: 10.1136/annrheumdis-2017-211468.]
2. Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Garcia-De La Torre I, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland). 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. Arthritis Rheumatol. 2017 Dec;69(12):2271-2282. doi: 10.1002/art.40320. [Abstract Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Garcia-De La Torre I, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project Consortium, the Euromyositis Register, and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (UK and Ireland). 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. Arthritis Rheumatol. 2017 Dec;69(12):2271-2282. doi: 10.1002/art.40320.]
3. Bottai M, Tjärnlund A, Santoni G, Werth VP, Pilkington C, de Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Danko K, Dimachkie MM, Feldman BM, García-De La Torre I, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinka M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Wook Song Y, Vencovsky J, Ytterberg SR, Miller FW, Rider LG, Lundberg IE; International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report. RMD Open. 2017 Nov 14;3(2):e000507. doi: 10.1136/rmdopen-2017-000507. eCollection 2017. [Abstract Bottai M, Tjärnlund A, Santoni G, Werth VP, Pilkington C, de Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Danko K, Dimachkie MM, Feldman BM, García-De La Torre I, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinka M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Wook Song Y, Vencovsky J, Ytterberg SR, Miller FW, Rider LG, Lundberg IE; International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report. RMD Open. 2017 Nov 14;3(2):e000507. doi: 10.1136/rmdopen-2017-000507. eCollection 2017.]

IMACS Project VII. Development of an Internationally Agreed Minimal Dataset for Juvenile Dermatomyositis (JDM) for Clinical and Research Use

Primary Objectives: 
To develop consensus on minimal elements to include in a JDM dataset to be used for clinical and research purposes.

Start Date: 2014
Completion Date: 2017

Lead Investigators:

Liza McCann

[email protected]

Publications:

1. McCann LJ, Pilkington CA, Huber AM, Ravelli A, Appelbe D, Kirkham JJ, Williamson PR, Aggarwal A, Christopher-Stine L, Constantin T, Feldman BM, Lundberg I, Maillard S, Mathiesen P, Murphy R, Pachman LM, Reed AM, Rider LG, van Royen-Kerkof A, Russo R, Spinty S, Wedderburn LR, Beresford MW. Development of a consensus core dataset in juvenile dermatomyositis for clinical use to inform research. Ann Rheum Dis. 2018 Feb;77(2):241-250. doi: 10.1136/annrheumdis-2017-212141. Epub 2017 Oct 30. [Abstract McCann LJ, Pilkington CA, Huber AM, Ravelli A, Appelbe D, Kirkham JJ, Williamson PR, Aggarwal A, Christopher-Stine L, Constantin T, Feldman BM, Lundberg I, Maillard S, Mathiesen P, Murphy R, Pachman LM, Reed AM, Rider LG, van Royen-Kerkof A, Russo R, Spinty S, Wedderburn LR, Beresford MW. Development of a consensus core dataset in juvenile dermatomyositis for clinical use to inform research. Ann Rheum Dis. 2018 Feb;77(2):241-250. doi: 10.1136/annrheumdis-2017-212141. Epub 2017 Oct 30.]
2. McCann LJ, Kirkham JJ, Wedderburn LR, Pilkington C, Huber AM, Ravelli A, Appelbe D, Williamson PR, Beresford MW.  Development of an internationally agreed minimal dataset for juvenile dermatomyositis (JDM) for clinical and research use.  Trials. 2015 Jun 12;16:268. [Abstract McCann LJ, Kirkham JJ, Wedderburn LR, Pilkington C, Huber AM, Ravelli A, Appelbe D, Williamson PR, Beresford MW.  Development of an internationally agreed minimal dataset for juvenile dermatomyositis (JDM) for clinical and research use.  Trials. 2015 Jun 12;16:268.]
3. McCann LJ, Arnold K, Pilkington CA, Huber AM, Ravelli A, Beard L, Beresford MW, Wedderburn LR. Developing a provisional, international Minimal Dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research. Pediatr Rheumatol Online J. 2014 Jul 21;12:31. [Abstract McCann LJ, Arnold K, Pilkington CA, Huber AM, Ravelli A, Beard L, Beresford MW, Wedderburn LR. Developing a provisional, international Minimal Dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research. Pediatr Rheumatol Online J. 2014 Jul 21;12:31.]

IMACS Project VIII. IMACS Outcomes Data Repository

Primary Objectives:
Develop a repository of databases of myositis natural history studies and therapeutic trials that have all collected the IMACS disease activity and damage core set measures, as well as core demographic and clinical data, for use by myositis researchers.

For further information on the IMACS Outcomes Data Repository, including use of the data that has been deposited, please see: IMACS Outcomes Repository Data Use Guidelines .

Start Date: 2004
Completion Date: Ongoing

Lead Investigators: 

Lisa G. Rider, M.D.
Head and Senior Clinician

Tel 301-451-6272
[email protected]

NIH Intramural Profile Lisa G. Rider, M.D.

10 CENTER DR

NIHBC 10 - CRC - HATFIELD

Bethesda, MD 20892-1301

Frederick W. Miller, M.D., Ph.D. (Retired)
Scientist Emeritus

Tel 984-287-3593
[email protected]

NIH Intramural Profile Frederick W. Miller, M.D., Ph.D. (Retired)

111 TW ALEXANDER DR

David P Rall Building

Research Triangle Park, NC 27709

Delivery Instructions

Publications: 

1. Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Magalhaes CS, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):911-923. doi: 10.1002/art.40060. [Abstract]
2. Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):898-910. doi: 10.1002/art.40064. [Abstract]
3. Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Saad Magalhaes C, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):782-791. doi: 10.1136/annrheumdis-2017-211401. [Abstract]
4. Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):792-801. doi: 10.1136/annrheumdis-2017-211400. [Abstract]
5. Rider LG, Ruperto N, Pistorio A, Erman B, Bayat N, Lachenbruch PA, Rockette H, Feldman BM, Huber AM, Hansen P, Oddis CV, Lundberg IE, Amato AA, Chinoy H, Cooper RG, Chung L, Danko K, Fiorentino D, García-De la Torre I, Reed AM, Wook Song Y, Cimaz R, Cuttica RJ, Pilkington CA, Martini A, van der Net J, Maillard S, Miller FW, Vencovsky J, Aggarwal R; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects. Rheumatology (Oxford). 2017. 56(11):1884-1893. doi: 10.1093/rheumatology/kex226. [Abstract]
6. Saygin D, Kim H, Douglas C, Erman B, Wilkerson J, McGrath JA, Oddis CV, Lundberg IE, Amato AA, García-De La Torre I, Chinoy H, Fiorentino D, Chung L, Song YW, Miller FW, Ruperto N, Vencovsky J, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS). Performance of the 2016 ACR-EULAR myositis response criteria in adult dermatomyositis/polymyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023. kead110. doi: 10.1093/rheumatology/kead110. [Abstract]
7. Kim H, Saygin D, Douglas C, Wilkerson J, Erman B, Pistorio A, McGrath JA, Reed AM, Oddis CV, Bracaglia C, van Royen-Kerkhof A, Bica B, Dolezalova P, Ferriani VPL, Flato B, Bernard-Medina AG, Herlin T, Miller FW, Vencovsky J, Ruperto N, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS) and Paediatric Rheumatology INternational Trials Organization (PRINTO). Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023 Mar 17:kead111. doi: 10.1093/rheumatology/kead111. Epub ahead of print. [Abstract]

IMACS Project IX. Standards of Treatment for Adults with Myositis and different Phenotypes – STAMP

Primary Objectives: 
To define standard approaches to treat myositis phenotypes.

Start Date: 2011
Completion Date: 2018

Lead Investigators:

Lisa Christopher-Stine, M.D., M.P.H.

[email protected]

Hector Chinoy, Ph.D., FRCP
Professor of Rheumatology & Neuromuscular Disease, Honorary Consultant Rheumatologist

[email protected]

Neil McHugh

[email protected]

Sarah Tansley

[email protected]

Lyubo Dourmishev

[email protected]

Publications:

1. Tansley S, Shaddick G, Christopher-Stine L, Sharp C, Dourmishev L, Maurer B, Chinoy H, McHugh N. Developing standardised treatment for adults with myositis and different phenotypes: an international survey of current prescribing preferences. Clin Exp Rheumatol 2016 May 31. Erratum in: Clin Exp Rheumatol. 2016 Nov-Dec;34(6):1127. [Abstract Tansley S, Shaddick G, Christopher-Stine L, Sharp C, Dourmishev L, Maurer B, Chinoy H, McHugh N. Developing standardised treatment for adults with myositis and different phenotypes: an international survey of current prescribing preferences. Clin Exp Rheumatol 2016 May 31. Erratum in: Clin Exp Rheumatol. 2016 Nov-Dec;34(6):1127.]

IMACS Project X. ACR-EULAR Project to Revise the Definition of Improvement and Major Clinical Response for Adult and Juvenile Dermatomyositis and Adult Polymyositis

Primary Objectives:
To develop new response criteria for adult and juvenile dermatomyositis and polymyositis, including criteria for minimal and major response for use as endpoints in myositis therapeutic trials. New response criteria have been developed and validated and endorsed by the American College of Rheumatology and European League Against Rheumatism (see Myositis Response Criteria). In a future phase of the work, the new criteria will be further refined and re-validated using a new clinical trials and studies.

Start Date: 2012
Completion Date: March 2023

Lead Investigators:

Lisa G. Rider, M.D.
Head and Senior Clinician

Tel 301-451-6272
[email protected]

NIH Intramural Profile Lisa G. Rider, M.D.

10 CENTER DR

NIHBC 10 - CRC - HATFIELD

Bethesda, MD 20892-1301

Nicola Ruperto

[email protected]

Frederick W. Miller, M.D., Ph.D. (Retired)
Scientist Emeritus

Tel 984-287-3593
[email protected]

NIH Intramural Profile Frederick W. Miller, M.D., Ph.D. (Retired)

111 TW ALEXANDER DR

David P Rall Building

Research Triangle Park, NC 27709

Delivery Instructions

Jiri Vencovsky

[email protected]

Rohit Aggarwal, M.D.

[email protected]

Publications:

1. Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Magalhaes CS, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):911-923. doi: 10.1002/art.40060. [Abstract Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Magalhaes CS, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):911-923. doi: 10.1002/art.40060.]
2. Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):898-910. doi: 10.1002/art.40064. [Abstract Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017. 69(5):898-910. doi: 10.1002/art.40064.]
3. Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Saad Magalhaes C, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):782-791. doi: 10.1136/annrheumdis-2017-211401. [Abstract Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM, Huber AM, Cimaz R, Cuttica RJ, de Oliveira SK, Lindsley CB, Pilkington CA, Punaro M, Ravelli A, Reed AM, Rouster-Stevens K, van Royen-Kerkhof A, Dressler F, Saad Magalhaes C, Constantin T, Davidson JE, Magnusson B, Russo R, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J, Ruperto N; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):782-791. doi: 10.1136/annrheumdis-2017-211401.]
4. Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):792-801. doi: 10.1136/annrheumdis-2017-211400. [Abstract Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017. 76(5):792-801. doi: 10.1136/annrheumdis-2017-211400.]
5. Rider LG, Ruperto N, Pistorio A, Erman B, Bayat N, Lachenbruch PA, Rockette H, Feldman BM, Huber AM, Hansen P, Oddis CV, Lundberg IE, Amato AA, Chinoy H, Cooper RG, Chung L, Danko K, Fiorentino D, García-De la Torre I, Reed AM, Wook Song Y, Cimaz R, Cuttica RJ, Pilkington CA, Martini A, van der Net J, Maillard S, Miller FW, Vencovsky J, Aggarwal R; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects. Rheumatology (Oxford). 2017. 56(11):1884-1893. doi: 10.1093/rheumatology/kex226. [Abstract Rider LG, Ruperto N, Pistorio A, Erman B, Bayat N, Lachenbruch PA, Rockette H, Feldman BM, Huber AM, Hansen P, Oddis CV, Lundberg IE, Amato AA, Chinoy H, Cooper RG, Chung L, Danko K, Fiorentino D, García-De la Torre I, Reed AM, Wook Song Y, Cimaz R, Cuttica RJ, Pilkington CA, Martini A, van der Net J, Maillard S, Miller FW, Vencovsky J, Aggarwal R; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects. Rheumatology (Oxford). 2017. 56(11):1884-1893. doi: 10.1093/rheumatology/kex226.]
6. Saygin D, Kim H, Douglas C, Erman B, Wilkerson J, McGrath JA, Oddis CV, Lundberg IE, Amato AA, García-De La Torre I, Chinoy H, Fiorentino D, Chung L, Song YW, Miller FW, Ruperto N, Vencovsky J, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS). Performance of the 2016 ACR-EULAR myositis response criteria in adult dermatomyositis/polymyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023. kead110. doi: 10.1093/rheumatology/kead110. [Abstract Saygin D, Kim H, Douglas C, Erman B, Wilkerson J, McGrath JA, Oddis CV, Lundberg IE, Amato AA, García-De La Torre I, Chinoy H, Fiorentino D, Chung L, Song YW, Miller FW, Ruperto N, Vencovsky J, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS). Performance of the 2016 ACR-EULAR myositis response criteria in adult dermatomyositis/polymyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023. kead110. doi: 10.1093/rheumatology/kead110.]
7. Kim H, Saygin D, Douglas C, Wilkerson J, Erman B, Pistorio A, McGrath JA, Reed AM, Oddis CV, Bracaglia C, van Royen-Kerkhof A, Bica B, Dolezalova P, Ferriani VPL, Flato B, Bernard-Medina AG, Herlin T, Miller FW, Vencovsky J, Ruperto N, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS) and Paediatric Rheumatology INternational Trials Organization (PRINTO). Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023 Mar 17:kead111. doi: 10.1093/rheumatology/kead111. Epub ahead of print. [Abstract Kim H, Saygin D, Douglas C, Wilkerson J, Erman B, Pistorio A, McGrath JA, Reed AM, Oddis CV, Bracaglia C, van Royen-Kerkhof A, Bica B, Dolezalova P, Ferriani VPL, Flato B, Bernard-Medina AG, Herlin T, Miller FW, Vencovsky J, Ruperto N, Aggarwal R, Rider LG; International Myositis Assessment and Clinical Studies Group (IMACS) and Paediatric Rheumatology INternational Trials Organization (PRINTO). Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles. Rheumatology (Oxford). 2023 Mar 17:kead111. doi: 10.1093/rheumatology/kead111. Epub ahead of print.]

IMACS Project XI. Guidelines for Cancer Screening and Follow-up of Myositis Phenotypes

Primary Objectives: 
Objectives of this project include derivation/definition of consensus guidelines for cancer screening in patients diagnosed with idiopathic inflammatory myopathies.

Start Date: 2015
Completion Date: 2023

Lead Investigators: 

Rohit Aggarwal, M.D.

[email protected]

Publications:

1. Oldroyd AGS, Allard AB, Callen JP, Chinoy H, Chung L, Fiorentino D, George MD, Gordon P, Kolstad K, Kurtzman DJB, Machado PM, McHugh NJ, Postolova A, Selva-O'Callaghan A, Schmidt J, Tansley S, Vleugels RA, Werth VP, Aggarwal R. A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies. Rheumatology (Oxford). 2021 Nov 3;60(11):5483. doi:10.1093/rheumatology/keab166. [Abstract Oldroyd AGS, Allard AB, Callen JP, Chinoy H, Chung L, Fiorentino D, George MD, Gordon P, Kolstad K, Kurtzman DJB, Machado PM, McHugh NJ, Postolova A, Selva-O'Callaghan A, Schmidt J, Tansley S, Vleugels RA, Werth VP, Aggarwal R. A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies. Rheumatology (Oxford). 2021 Nov 3;60(11):5483. doi:10.1093/rheumatology/keab166.] 
2. Oldroyd AGS, Callen JP, Chinoy H, Chung L, Fiorentino D, Gordon P, Machado P, McHugh N, Selva-O’Callaghan A, Schmidt J, Tansley SL, Vleugels RA, Werth VP, International Myositis Assessment and Clinical Studies Group Cancer Screening Expert Group*, and Aggarwal R. International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening An International Myositis Assessment and Clinical Studies Group (IMACS) Initiative. Nature Reviews Rheumatol. 2023, In press.

IMACS Project XII. Screening, Treatment, and Monitoring of Myositis-associated Interstitial Lung Disease

Primary Objectives:  
Through the development of a combined retrospective/prospective database, the purpose of this project is to devise consensus guidelines for diagnosis, management, and outcomes assessment in myositis-associated ILD.

Start Date: 2015
Completion Date: 2023

Lead Investigators:

Dana Ascherman, M.D.

[email protected]

Publications:
None

IMACS Project XIII. The Role of B Cell Depletion Therapy in the Inflammatory Myopathies

Primary Objectives: 
This purpose of this project is to establish a registry of myositis patients using rituximab in myositis and related conditions, with goal of a minimal data collection, including details on the use of rituximab and clinical responses. Goals of the registry include learning more about the use of B-cell depletion in the treatment of the inflammatory muscle diseases, including the speed with which it is likely to work, whether it does work as well early in disease as later in disease, whether there is the potential to reduce steroid use substantially, whether the biosimilars for Rituximab and the fully humanized anti-CD20 biologics are as successful, and how long the beneficial effects of B-cell depletion are likely to last.

Start Date: 2021
Completion Date: 2023

Lead Investigators:

David A. Isenberg, M.D., FRCP

[email protected]

Publications:

1. Oliveira T, Yildirim R, Deakin C, Isenberg D. Rituximab in myositis: where are we now? A survey of current usage. Rheumatology (Oxford). 2023 Jul 5;62(7):e211-e212. doi: 10.1093/rheumatology/kead010. [Abstract Oliveira T, Yildirim R, Deakin C, Isenberg D. Rituximab in myositis: where are we now? A survey of current usage. Rheumatology (Oxford). 2023 Jul 5;62(7):e211-e212. doi: 10.1093/rheumatology/kead010.]

IMACS Project XIV. An Educational Computer Program for Physicians Caring for Adult IIM Patients

Primary Objectives:
Most rheumatologists and neurologists see only a few IIM patients each year, and treatment decisions are difficult, as each one likely differs from patients typically included in therapeutic trials. We have created an educational tool that allows a physician “opinion-seeker“ to obtain feedback on how his/her peers and IIM experts would treat a similar patient with IIM. A web-based database collects data from templates about given patients, their clinical and laboratory features, and therapies received. The data is translated into patient narratives and will be subsequently circulated to “opinion-givers” who also use templates to provide their suggestions about changes in treatment. The opinions provided on these templates will then be summarized to show how various treatment suggestions are distributed among the opinion-givers. The opinions can be subdivided into all respondents or those who are experts in the field. The data collected may also facilitate research into the kinds of treatment being received for patients with varying presentations of the disease.

Start Date: 2018
Completion Date: Not Active

Lead Investigators:

Murray Baron, M.D.

[email protected]

Publications:

None

IMACS Project XVIII. Defining Myositis Phenotypes and Therapies that Achieve Complete Clinical Responses and Remission

Primary Objectives: 
To define which therapies are useful in which phenotypes to achieve complete clinical responses and remission.

Start Date: 2011
Completion Date: Ongoing

Lead Investigators: 

Mazen Dimachkie, M.D.
Professor of Neurology & Director of Neuromuscular Division, Executive Vice Chairman for Research Programs, Department of Neurology

[email protected]

University of Kansas Medical Center

Kansas City, Kansas, USA

Ann Reed, M.D.
Professor and Chair, Department of Pediatrics, Physician-in-Chief, Duke Children's Hospital

Tel 919-681-4080
[email protected]

Duke University Medical Center

Box 3352

Durham, NC 27710

Chet Oddis

[email protected]

Hector Chinoy, Ph.D., FRCP
Professor of Rheumatology & Neuromuscular Disease, Honorary Consultant Rheumatologist

[email protected]

Rohit Aggarwal, M.D.

[email protected]

David A. Isenberg, M.D., FRCP

[email protected]

Ingrid Lundberg, M.D., Ph.D.

Tel 011-46-8-5177-3080
[email protected]

Rheumatology Unit

Department of Medicine

Karolinska University Hospital

Solna, Karolinska Institutet

SE- 171 76 Stockholm, Sweden

Publications: 
None

IMACS Project XVIII-B. Critical Evaluation of Available Outcome Measures for IBM and Development and Validation of a Core Set of Functional Measures for IBM

Primary Objectives:

Specific Aim 1:
To identify all existing functional outcome measures in inclusion body myositis. The usefulness and limitations of the tools, and their utility for use in clinical care and/or clinical trials will be determined.

Specific Aim 2:
To identify strength and functional outcome measures used in other idiopathic inflammatory myopathies and neuromuscular diseases which could potentially be repurposed for use in inclusion body myositis. The usefulness and limitations of the the tools, and their utility for use in clinical care and/or clinical trials will be determined

Specific Aim 3:
Use a consensus building approach to establish a core set of strength and functional measures for inclusion body myositis.

Start Date: 2021
Completion Date: Ongoing

Lead Investigators:

Bhaskar Roy

[email protected]

Lindsay Alfano

[email protected]

Publications:

1. Roy B, Lucchini M, Lilleker JB, Goyal NA, Naddaf E, Adler B, Alfano LN, Malandraki GA, Focht Garand KL, Mochel D, Badrising U, Machado PM, Pagkatipunan R, Ramdharry G, Wang L, Funaro MC, Schmidt J, Kushlaf H, Schiopu E, Stipancic K, Goyal N, d'Alessandro M, Conticini E, Cruz-Coble B, Lloyd TE; International Myositis Assessment and Clinical Studies (IMACS) Inclusion Body Myositis Scientific Interest Group. Current status of clinical outcome measures in inclusion body myositis: a systematised review. Clin Exp Rheumatol. 2023 Mar;41(2):370-378. doi: 10.55563/clinexprheumatol/ifacv3. Epub 2023 Feb 3. [Abstract Roy B, Lucchini M, Lilleker JB, Goyal NA, Naddaf E, Adler B, Alfano LN, Malandraki GA, Focht Garand KL, Mochel D, Badrising U, Machado PM, Pagkatipunan R, Ramdharry G, Wang L, Funaro MC, Schmidt J, Kushlaf H, Schiopu E, Stipancic K, Goyal N, d'Alessandro M, Conticini E, Cruz-Coble B, Lloyd TE; International Myositis Assessment and Clinical Studies (IMACS) Inclusion Body Myositis Scientific Interest Group. Current status of clinical outcome measures in inclusion body myositis: a systematised review. Clin Exp Rheumatol. 2023 Mar;41(2):370-378. doi: 10.55563/clinexprheumatol/ifacv3. Epub 2023 Feb 3.]

IMACS Project XX. An International Survey of Muscle Biopsy Practices and Experiences in the Evaluation of Myositis

Primary Objectives:

Muscle biopsy is a commonly performed procedure in patients with suspected myositis, however muscle biopsy practices and techniques vary widely between institutions. Additionally, little is known about the experience of muscle biopsy procedures for patients with myositis. The purpose of this study is to explore:

1.  the experiences of clinicians who request or perform muscle biopsies in patients with myositis.
2. the experiences of patients with myositis with respect to muscle biopsy. The information obtained may ultimately help inform recommendations regarding muscle biopsy practices in myositis.

Start Date: 10/21/2022
Completion Date: Ongoing

Lead Investigators:

Jessica Day, Ph.D., MBBS, FRACP

[email protected]

Publications:

1. Maxwell S, Ross L, Oon S, Wicks IP, Day J. Muscle biopsy practices in the evaluation of idiopathic inflammatory myopathies: An international survey of expert clinicians. Semin Arthritis Rheum. 2024 Oct;68:152519. doi: 10.1016/j.semarthrit.2024.152519. Epub 2024 Jul 15. PMID: 39059155. [Abstract]
2. Sutu B, Maxwell S, Oon S, Ross L, Day J. Patient experiences of muscle biopsy in idiopathic inflammatory myopathies: a cross-sectional survey. Rheumatol Int. 2024 Oct;44(10):2129-2137. doi: 10.1007/s00296-024-05668-4. Epub 2024 Jul 31. PMID: 39083047; PMCID: PMC11393206. [Abstract]