SEARCH

SEARCH BY CITATION

Abstract

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

Objective

To use consensus methods and the considerable expertise contained within the Childhood Arthritis and Rheumatology Research Alliance (CARRA) organization to extend the 3 previously developed treatment plans for moderate juvenile dermatomyositis (DM) to span the full course of treatment.

Methods

A consensus meeting was held in Chicago on April 23–24, 2010, involving 30 pediatric rheumatologists and 4 lay participants. Nominal group technique was used to achieve consensus on treatment plans that represented typical management of moderate juvenile DM. A preconference survey of CARRA, completed by 151 (56%) of 272 members, was used to provide additional guidance to the discussion.

Results

Consensus was reached on timing and rate of steroid tapering, duration of steroid therapy, and actions to be taken if patients were unchanged, worsening, or experiencing medication side effects or disease complications. Of particular importance, a single consensus steroid taper was developed.

Conclusion

We were able to develop consensus treatment plans that describe therapy for moderate juvenile DM throughout the treatment course. These treatment plans can now be used clinically, and data collected prospectively regarding treatment effectiveness and toxicity. This will allow comparison of these treatment plans and facilitate the development of evidence-based treatment recommendations for moderate juvenile DM.


INTRODUCTION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

Juvenile dermatomyositis (DM) is a rare autoimmune illness characterized by muscle and skin involvement, with less frequent involvement of other systems, including the gastrointestinal tract and lungs. Although previously associated with significant mortality (1), morbidity is a much greater problem since the introduction of corticosteroids as a mainstay of therapy. Many children experience complications of their underlying disease, such as contractures, weakness, disfiguring skin lesions, and painful calcinosis. Many children may also develop complications secondary to prolonged courses of treatment, in particular, those side effects related to prolonged corticosteroid use (2).

There are very little data on which to base treatment decisions in juvenile DM. Even the most commonly used therapies, corticosteroids and/or methotrexate, have not been studied in clinical trials. In fact, at the time this article was written, no randomized clinical trials of therapy in juvenile DM had been published. This lack of data has resulted in wide variation in treatment of children with juvenile DM. This has been documented by Stringer et al, who reported the results of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) juvenile DM treatment survey (3). Using a series of representative clinical scenarios of children with juvenile DM, members of CARRA were surveyed with regard to investigations and therapies. The survey was answered by 141 CARRA members (84% response rate) and revealed a remarkable degree of variation in dose, duration and route of corticosteroids, and use of methotrexate and other immunosuppressive agents (3).

In 2007, 12 pediatric rheumatologists with experience in the care of children with juvenile DM participated in a CARRA-initiated consensus conference in Toronto, Ontario, Canada (4). This meeting had the explicit goal of defining a small number of consensus treatment plans for the initial treatment (up to 2 months) of children with moderate juvenile DM. Using data from the CARRA juvenile DM treatment survey (3) and expert opinions, the meeting participants were able to develop 3 consensus treatment plans. It is important to note that these treatment plans were not intended to be innovative; rather, the goal was to develop treatment plans that were similar to approaches that were being used commonly in the pediatric rheumatology community. It was hoped that by developing consensus treatment plans, variation in treatment approaches could be decreased and data could be prospectively collected. This would allow for comparative research, and would be the first step in defining evidence-based treatments for moderate juvenile DM.

After the success of the Toronto consensus meeting, it was recognized that for these treatment plans to be studied, they needed to be extended beyond 2 months of treatment. The goal of the present report was to use consensus methods, and the considerable expertise contained within the CARRA organization, to extend the 3 previously developed treatment plans to span the full course of treatment of children with moderate juvenile DM.

Significance & Innovations

  • We have successfully identified consensus treatment plans for moderate juvenile dermatomyositis that reflect commonly used treatment approaches.

  • These treatment plans include consensus on corticosteroid weaning.

  • This is an important step in the development of evidence-based treatment recommendations for moderate juvenile dermatomyositis.

MATERIALS AND METHODS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

CARRA.

CARRA is a North American organization of pediatric rheumatologists whose mission is to prevent, treat, and cure rheumatic diseases in children and adolescents through fostering, facilitating, and conducting high-quality research. CARRA currently has more than 304 members from 92 centers, and includes the majority of pediatric rheumatologists in North America.

Premeeting survey.

Prior to the consensus meeting, which was held during the 2010 annual CARRA meeting, an electronic survey was sent to all CARRA members. This survey collected information about treatment strategies beyond the initial 2 months and what information respondents used to make treatment decisions. Several options were provided for each question, with an open text field available for each question (treatment-related questions from this survey are listed in Supplementary Appendix A, available in the online version of this article at http://onlinelibrary.wiley.com/journal/10.1002/(ISSN)2151-4658). In total, 151 (56%) of 272 CARRA members responded to the questionnaire. The results were used to inform discussion during the consensus meeting.

On average, the respondents to this survey graduated from medical school in 1991 (range 1961–2006, SD 11 years), were 61% women (n = 92), had a mean ± SD age of 45.2 ± 10.5 (range 28–73 years), and were actively managing a median of 8 patients (range 0–210, 25th percentile 5, 75th percentile 15) with juvenile DM at the time of the survey.

Consensus meeting.

The 2-day consensus meeting (April 23–24, 2010) was attended by 30 pediatric rheumatologists, one of whom acted as facilitator (AMH). The facilitator participated in all discussions, but did not vote. There were also 4 lay participants with particular interest and/or experience with juvenile DM who participated in discussions, but did not vote (see Appendix A for a list of the meeting participants).

Despite the challenge imposed by the large number of meeting participants, nominal group technique was used to achieve consensus for all questions considered during the meeting. The same format was used for each question. The facilitator framed the question to be discussed, and then showed data from the survey relevant to that question. Each participant then had an opportunity to express their opinion for 1–2 minutes without interruption. Potential responses to the question were recorded on flip charts at the front of the room, and detailed notes were kept. Participants were then given the opportunity to vote for their preferred responses to the questions. Each participant had 5 stickers that they attached to the flip charts; stickers could be distributed in any way (e.g., all 5 on 1 answer or 1 sticker each for 5 answers). If there was a clear preferred response, this was considered to be the consensus answer to the question. If not, participants were once again given the opportunity to speak uninterrupted for 1–2 minutes. After discarding answers that clearly were not preferred, there was another round of voting by each participant with another 5 stickers. This process continued until there was a clear consensus or a stalemate was reached. At some points, if the choices had been reduced to 2, a show-of-hands vote was conducted if all participants agreed to an open vote. Consensus was defined as at least 80%.

The process was dynamic, with discussion and decisions from earlier in the meeting influencing the questions discussed and the overall goals as the meeting progressed. In fact, the overall approach to the goal of extending the treatment plans beyond 2 months changed during the meeting. It became clear on the first day of the meeting that the most important component of the extended treatment plan was the approach to the corticosteroid taper. Initially, it was intended that the corticosteroid taper would be based on the answers to the following questions: 1) when should the first taper of prednisone be attempted, assuming patient has met improvement criteria?, 2) at what interval should corticosteroids be tapered?, and 3) by what dose should prednisone be tapered at each step?

During the first day, it became clear that there were some logistic issues with this approach. For example, some proposed tapering protocols resulted in differences in corticosteroid duration based on body weight. As discussion progressed on the first day, most consensus participants also realized that they were more interested in total time on corticosteroids than in the tapering details. It was then agreed, unanimously, that the corticosteroid taper would be based on achieving targets (e.g., reaching certain percentages of the starting corticosteroid dose at specified times, such as 75% of the starting dose by time 1).

The questions that were considered during this consensus meeting are summarized in Table 1. Table 2 shows the patient characteristics considered to be consistent with moderate juvenile DM, and Table 3 shows the initial 2-month treatments, as decided during the Toronto consensus meeting (4).

Table 1. Questions and results considered during the consensus meeting*
 QuestionMethodResponsesVotes
  • *

    NA = not applicable.

  • Only responses with substantial support are included.

1 (initial vote)What criteria should be used to decide that a patient has improved and a taper can proceed?Sticker votePhysician judgment based on core set (improvement/unacceptable toxicity)50
Strength improved or normal23
1 (final vote)What criteria should be used to decide that a patient has improved and a taper can proceed?Hand votePatient improved or unacceptable toxicity (defined as strength improved or normal, enzymes improved or normal, rash stable/improved/absent, and additional criteria consistent with improvement)25
Physician judgment based on core set improvement or unacceptable toxicity1
2 (initial vote)When should first taper of prednisone be attempted (assuming patient has met improvement criteria)?Sticker vote4 weeks60
When improvement criteria are first met48
2 (final vote)When should first taper of prednisone be attempted (assuming patient has met improvement criteria)?Hand vote4 weeks20
When improvement criteria are first met4
3 (initial vote)At what interval should prednisone be tapered?Sticker voteEvery 2 weeks from 2 to 0.5 mg/kg, then every 4 weeks43
Every 2–4 weeks25
Every 4 weeks, then when less than 0.2 mg/kg every 8 weeks24
Every 2 weeks20
3 (second vote)At what interval should prednisone be tapered?Sticker voteEvery 2 weeks at 2 to 0.5 mg/kg, then every 4 weeks66
Every 2 weeks29
Every 4 weeks at 2.0 to 0.2 mg/kg, then every 8 weeks28
3 (final vote)At what interval should prednisone be tapered?Hand voteEvery 2 weeks at 2 to 0.5 mg/kg, then every 4 weeks24
Disagree3
4 (initial vote)By what dose should prednisone be tapered at each step?Sticker vote20% of current dose56
10% of current dose38
0.25 mg/kg × 4, 0.125 mg/kg × 4, then 0.05 mg/kg × 4 to 5 mg, then off corticosteroid15
5When should children come off corticosteroids?No voting4–8 monthsNA
>12 monthsNA
6What criteria should be used to decide that a patient is unchanged?Hand voteNot improved and not worse (physician judgment)19
Physician judgment1
Physician and parent judgment (combined)1
7 (initial vote)What should be done when a patient meets criteria for being unchanged (assuming patient is not in remission)?Sticker voteHold dose for 4 weeks, then escalate therapy if still unchanged45
Physician judgment38
7 (final vote)What should be done when a patient meets criteria for being unchanged (assuming patient is not in remission)?Hand voteHold dose for 4 weeks, then escalate therapy if still unchanged14
 Physician judgment3
Table 2. Patient characteristics for moderate juvenile DM*
  • *

    DM = dermatomyositis; CMAS = Childhood Myositis Assessment Scale; MMT8 = Manual Muscle Testing, 8-muscle group.

Patients should have
 Rash (Gottron's rash, heliotrope rash, or extensor surface rash)
 Muscle weakness
 Evidence of myositis (by biopsy, magnetic resonance imaging, or electromyography)
 Age <16 years at onset
 Physician global assessment of moderate (on a 3-category scale of mild, moderate, or severe)
Patients should not have
 Severe disability as defined by cannot get out of bed, CMAS score <15, or MMT8 score <30
 Parenchymal lung disease
 Gastrointestinal vasculitis (as determined by imaging or presence of bloody stools)
 Other autoimmune or mimicking disease (as determined by the treating physician)
 Requirement for intensive care unit management
 Presence of aspiration or dysphagia to the point of inability to swallow
 Central nervous system disease (defined as decreased level of consciousness or seizures)
 Skin ulceration
 Medication contraindication
 Myocarditis
 Pregnancy
 Significant calcinosis (as determined by the treating physician)
 Age <1 year
Table 3. Initial treatment plans for the first 4 weeks
Treatment A
 Intravenous methylprednisolone
  30 mg/kg/day (maximum 1 gm) once a day for 3 days. May continue 1 × per week (optional)
 Methotrexate
  Subcutaneous unless only oral possible: lesser of 15 mg/m2 or 1 mg/kg (maximum 40 mg) once weekly
 Prednisone
  

2 mg/kg/day (maximum 60 mg) once daily × 4 weeks, then follow schedule in Table 4

Treatment B
 Intravenous methylprednisolone
  30 mg/kg/day (maximum 1 gm) once a day for 3 days. May continue 1 × per week (optional)
 Methotrexate
  Subcutaneous unless only oral possible: lesser of 15 mg/m2 or 1 mg/kg (maximum 40 mg) once weekly
 Prednisone
  

2 mg/kg/day (maximum 60 mg) once daily × 4 weeks, then follow schedule in Table 4

 Intravenous immunoglobulin
  2 gm/kg (maximum 70 gm), every 2 weeks × 3, then monthly (optional intravenous methylprednisolone × 1 with each dose)
Treatment C
 Methotrexate
  Subcutaneous unless only oral possible: lesser of 15 mg/m2 or 1 mg/kg (maximum 40 mg) once weekly
 Prednisone
  

2 mg/kg/day (maximum 60 mg) once daily × 4 weeks, then follow schedule in Table 4

Table 4. Consensus steroid tapering schedule*
 Any weight10 kg15 kg20 kg25 kg≥30 kg
  • *

    Treating physicians may use either the any weight column to calculate steroid targets or the weight column that is closest to the patient in question.

  • Percentages that were agreed upon to be used as targets.

0 weeks (100%)2 mg/kg2030405060
4 weeks 17.527.5354555
6 weeks  2532.54050
8 weeks (75%)1.5 mg/kg1522.53037.545
10 weeks 12.5202532.540
12 weeks  17.522.527.535
14 weeks (50%)1.0 mg/kg1015202530
16 weeks  12.517.52025
18 weeks 7.5101517.520
20 weeks   12.51517.5
22 weeks (25%)0.5 mg/kg57.51012.515
24 weeks   81012.5
26 weeks 45 7.510
28 weeks   6 7.5
30 weeks 3  5 
32 weeks  2.54 5
34 weeks 2    
36 weeks (10%)0.2 mg/kg 1.522.53
38 weeks 1    
40 weeks  1  2
42 weeks   1  
44 weeks    1 
46 weeks     1
50 weeks (0%)Off00000

RESULTS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

The first question that was discussed concerned what criteria could be used to determine that a patient was improved and the corticosteroid taper could begin. There was considerable discussion about the role of physician judgment and how to quantify this. It was agreed (25 of 26 votes, 96%) that, although physician judgment was needed to determine this point, it should be based on the following criteria: strength improved or normal, enzymes improved or normal, rash stable/improved/absent, and additional criteria consistent with improvement (not specifically defined, and could vary depending on patient and physician factors).

The second question concerned when corticosteroid tapering could begin, assuming improvement criteria were met. The discussion largely centered on whether tapering could begin at the first point where improvement was documented, or if one should wait until a certain amount of time has elapsed. Consensus was reached (20 of 24 votes, 83%) for waiting until 4 weeks of treatment (assuming improvement criteria were met).

The third question concerned the frequency of corticosteroid weaning. There was considerable variation in opinion during the first round of voting; however, in the ensuing discussion, several participants indicated that their choice for longer intervals was based on concerns that patients might not be ready for a dose reduction due to a variety of clinical or biochemical factors. When it was emphasized that weaning of corticosteroid would only occur when the physician thought it was appropriate, these participants indicated they would be comfortable with a shorter weaning period. After 2 further rounds of voting, consensus was reached (24 of 27 votes, 89%) on a weaning interval of every 2 weeks on corticosteroid doses from 2 to 0.5 mg/kg, and then every 4 weeks thereafter.

The fourth question addressed how much corticosteroid should be tapered at each step. This question resulted in considerable discussion. There were 2 main issues. First, it became clear that there were 2 groups among the participants: a group that favored an aggressive, faster tapering of corticosteroid (off of corticosteroid in 4–8 months) and another group that favored a slower taper (>12 months to discontinuation). Second, while the participants were willing to discuss the approach set out for the meeting (deciding on how often to wean and by how much), many were more comfortable with setting specific targets (how long to come off corticosteroids, how long to get to 1 mg/kg, etc.). Much of this discussion occurred near the end of the first day. On the second day, it was agreed that the approach would be changed, and that participants would attempt to reach consensus on weaning targets (unanimous). Question 4 was not discussed further.

Before proceeding to the next question, there was discussion about which percentages should be used as targets. It was agreed that the targets would be the following: 75%, 50%, 25%, 10%, and 0% of initial starting corticosteroid dose.

The fifth question concerned when children would come off corticosteroids, and formed the basis for establishing the steroid tapering schedule. It initially appeared that it would not be possible to reach consensus, as there were substantial numbers of participants who favored either a short or a long period of corticosteroid treatment. At this point, these 2 groups were separated and asked to independently develop tapering regimens. Despite not being instructed to do so, both groups developed very similar plans. There was unanimity that the facilitator would generate a corticosteroid weaning plan that summarized the 2 proposed plans. This consensus steroid weaning plan is shown in Table 4.

At this point, there was little time left in the meeting. The final discussions concerned how to define whether a patient's disease status was unchanged, and what actions to take if patients were unchanged on visits where corticosteroid tapering was planned. The results of these discussions are summarized in Table 1 (questions 6 and 7). Finally, participants agreed that other issues that were not discussed (e.g., what to do if patients are worsening, responses to drug toxicity or disease complications) would be left to the judgment of the treating physician. This was consistent with the results of the premeeting survey.

A summary of patient flow when using these treatment plans is shown in Figure 1. The complete treatment plans, including assessment recommendations, can be downloaded from the CARRA web site at www.carragroup.org.

thumbnail image

Figure 1. Patient flow diagram for consensus treatment plans. JDM = juvenile dermatomyositis; IVIG = intravenous immunoglobulin.

Download figure to PowerPoint

DISCUSSION

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

Through a consensus process, members of CARRA who participated in this meeting were able to develop consensus treatment plans that extend therapy beyond the initial 2 months. In combination with the results of the first consensus meeting (4), the resulting 3 complete treatment plans describe typical therapy for children with moderate juvenile DM from treatment initiation to discontinuation of corticosteroids. Considering the amount of variation in treatment approaches that has been previously documented (3), this is a remarkable achievement.

It is important to note that these treatment plans are not to be considered standard of care and are not innovative or cutting-edge therapy for moderate juvenile DM. These treatment plans will not be appropriate for all patients with moderate juvenile DM, depending on a variety of difficult-to-quantify factors. It is also possible that some patients will need to deviate from a treatment plan, depending on their disease course and the opinion of the treating physician. The intent was to develop treatment plans that were similar to the care that would commonly be provided by the majority of pediatric rheumatologists. This will limit treatment variation and facilitate prospective, meaningful comparisons between treatments, and assist in future development of evidence-based recommendations. We emphasize that these are not treatment recommendations. Approaches different from these treatment plans may be equally or more safe and effective. Given that these treatment plans are based on expert opinion of usual practice, they can be considered as representative of a low level of evidence (level 3, according to the Strength of Recommendation Taxonomy [5]).

Since these treatment plans are not intended as recommendations, the ultimate goal of developing them requires discussion and explanation. There have been no randomized clinical trials published for treatment of new-onset juvenile DM, related partly to the rarity of the illness and partly to the difficulties in assessing this complex illness. We note that there is an ongoing clinical trial involving corticosteroids, methotrexate, and cyclosporine in new-onset juvenile DM (http://www.printo.it/project_ongoing_detail.asp?ProjectID=14). However, it is unlikely that there will be substantial numbers of clinical trials in the future. This will continue to limit the development of evidence-based treatment recommendations in juvenile DM. For this reason, CARRA has chosen an alternative approach, called comparative effectiveness research (CER), to determine the best treatments in juvenile DM. CER includes powerful methods of evaluating therapies through analysis of prospectively collected data obtained during the provision of routine clinical care (6, 7). This approach is widely being used and is promoted by the National Institutes of Health, insurers, and policymakers as an alternative to the traditional clinical trial (8, 9). CER has significant advantages over randomized clinical trials in the areas of cost and efficiency, and, given that data are collected within the context of clinical care, it may be more generalizable.

Our intent is that the treating pediatric rheumatologists will be able to use a treatment plan that is similar to their usual practice. By doing so, variation will be reduced, and large numbers of children will be treated with one of these treatment plans. Prospective data collected on these patients can then be used for CER to determine which treatment approach is the most successful, with regard to both effectiveness and toxicity. Subsequent iterations of CER using updated and revised treatment plans will ultimately lead to determination of the optimal care for children with moderate juvenile DM. In the future, this methodology will be expanded to include other forms of juvenile DM (mild, severe, ulcerative, etc.) and to include other rheumatic illnesses.

The assessment of children with juvenile DM is challenging. Progress has been made in the description of core set assessments (10, 11) and preliminary definitions of improvement (12–14). These recommendations include multiple assessments, and are best suited to clinical trials with appropriate infrastructure support. Unfortunately, the combination of these assessments is probably not practical in the typical clinician's office. For this reason, we have previously recommended a reduced, minimum data collection to be used as part of the CER data collection, with the more detailed assessments left as an option (4).

There are some potential limitations to this report. Although the consensus meeting involved a large number of pediatric rheumatologists, there were likely some who would disagree with the decisions made. There was considerable discussion about some issues within our small group. We recognize that not all physicians will be able to identify a treatment plan that is similar to their usual care of moderate juvenile DM, and that it is impossible for a treatment plan to accommodate all possible courses that a patient may take. These treatment plans do not replace the clinical judgment of treating physicians. Also, while statistical methods that are intended to control for patient factors that influence treatment decisions exist, there is no guarantee that these methods will be able to completely eliminate bias in the comparison of these treatment plans.

In conclusion, we showed here the completed CARRA Consensus Treatments for children with new-onset moderate juvenile DM. This has been a collaborative effort of the investigators of CARRA, and as such should have a wide appeal and acceptability to pediatric rheumatologists across North America and beyond. The next steps are to prospectively collect data on patients treated with these plans as part of routine clinical care, and through an iterative, analytic process, identify the treatments with the best outcomes and least side effects for children with juvenile DM.

AUTHOR CONTRIBUTIONS

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. Dr. Huber had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Study conception and design. Huber, Feldman, Wallace.

Acquisition of data. Huber, Robinson, Reed, Abramson, Bout-Tabaku, Carrasco, Curran, Feldman, Gewanter, Griffin, Haines, Hoeltzel, Isgro, Kahn, Lang, Lawler, Shaham, Schmeling, Scuccimarri, Shishov, Stringer, Wohrley, Ilowite, Wallace.

Analysis and interpretation of data. Huber, Wohrley, Wallace.

Acknowledgements

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

Our sincere thanks to Drs. Lisa Rider, Lauren Pachman, and Dan Solomon for their valuable input into this project, and to Audrey Hendrickson and Vaishali Tenkale for their outstanding administrative assistance.

REFERENCES

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information
  • 1
    Bitnum S, Daeschner C Jr, Travis LB, Dodge WF, Hopps HC. Dermatomyositis. J Pediatr 1964; 64: 10131.
  • 2
    Huber AM, Lang B, LeBlanc CM, Birdi N, Bolaria R, Malleson P, et al. Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 2000; 43: 5419.
  • 3
    Stringer E, Ota S, Bohnsack J, Bowyer SL, Griffin TA, Huber AM, et al. Treatment approaches to juvenile dermatomyositis across North America: the Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM treatment survey. J Rheumatol 2009; 37: 195361.
  • 4
    Huber AM, Giannini EH, Bowyer SL, Kim S, Lang B, Lindsley CB, et al. Protocols for the initial treatment of moderately severe juvenile dermatomyositis: results of a Children's Arthritis and Rheumatology Research Alliance Consensus Conference. Arthritis Care Res (Hoboken) 2010; 62: 21925.
  • 5
    Ebell MH, Siwek J, Weiss BD, Woolf SH, Susman J, Ewigman B, et al. Strength of recommendation taxonomy (SORT): a patient-centered approach to grading evidence in the medical literature. Am Fam Physician 2004; 69: 54856.
  • 6
    Sox HC. Defining comparative effectiveness research: the importance of getting it right. Med Care 2010; 48 Suppl: S78.
  • 7
    Tunis SR, Benner J, McClellan M. Comparative effectiveness research: policy context, methods development and research infrastructure. Stat Med 2010; 29: 196376.
  • 8
    Sox HC. Comparative effectiveness research: a progress report. Ann Intern Med 2010; 153: 46972.
  • 9
    Luce BR, Kramer JM, Goodman SN, Connor JT, Tunis S, Whicher D, et al. Rethinking randomized clinical trials for comparative effectiveness research: the need for transformational change. Ann Intern Med 2009; 151: 2069.
  • 10
    Ruperto N, Ravelli A, Murray K, Lovell DJ, Andersson-Gare B, Feldman BM, et al. Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis. Rheumatology (Oxford) 2003; 42: 14529.
  • 11
    Miller FM, Rider LG, Chung Y, Cooper R, Danko K, Farewell V, et al. Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathy. Rheumatology (Oxford) 2001; 40: 126273.
  • 12
    Ruperto N, Pistorio A, Ravelli A, Rider LG, Pilkington C, Oliveira S, et al. The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62: 153341.
  • 13
    Rider LG, Giannini EH, Brunner HI, Ruperto N, James-Newton L, Reed AM, et al. International consensus on preliminary definitions of improvement in adult and juvenile myositis. Arthritis Rheum 2004; 50: 228190.
  • 14
    Rider LG, Giannini EH, Harris-Love M, Joe G, Isenberg D, Pilkington C, et al. Defining clinical improvement in adult and juvenile myositis. J Rheumatol 2003; 30: 60317.

APPENDIX A

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

CHICAGO CONSENSUS MEETING PARTICIPANTS

Members of the Juvenile Dermatomyositis Subcommittee of the Childhood Arthritis and Rheumatology Research Alliance who participated: Leslie Abramson, Barbara Adams, Sharon Bout-Tabaku, Ruy Carrasco, Megan Curran, Peter Dent, Barbara Edelheit, Brian Feldman, Adam Huber, Josephine Isgro, Harry Gewanter, Thomas Griffin, Kathleen Haines, Mark Hoeltzel, Philip Kahn, Dan Kingsbury, Ann Kunkel, Bianca Lang, Angela Robinson, Heinrike Schmeling, Kara Schmidt, Rosie Scuccimarri, Bracha Shaham, Michael Shishov, Elizabeth Stringer, Heather Van Mater, and Carol Wallace. Lay participants: John Hayhurst, Patti Lawler, Debbie Wright, and Julie Wohrley.

Supporting Information

  1. Top of page
  2. Abstract
  3. INTRODUCTION
  4. MATERIALS AND METHODS
  5. RESULTS
  6. DISCUSSION
  7. AUTHOR CONTRIBUTIONS
  8. Acknowledgements
  9. REFERENCES
  10. APPENDIX A
  11. Supporting Information

Additional Supporting Information may be found in the online version of this article.

FilenameFormatSizeDescription
ACR_20695_sm_SupplData.doc36KSupplementary Data

Please note: Wiley Blackwell is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.