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ORIGINAL ARTICLE
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 2324, 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.
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 asso-
INTRODUCTION
546
We have successfully identied consensus treatment plans for moderate juvenile dermatomyositis
that reect commonly used treatment approaches.
547
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
dening 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 rst step in dening evidencebased 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.
548
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 2324, 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 12 minutes without interruption. Potential responses to the question were recorded on ip 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 ip 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 12
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 dened as at
least 80%.
The process was dynamic, with discussion and decisions from earlier in the meeting inuencing 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 rst 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 rst 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 rst 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 rst day, most consensus participants
also realized that they were more interested in total time
Huber et al
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 specied
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).
RESULTS
The rst 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 dened, 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 rst 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 rst 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 specic targets (how
long to come off corticosteroids, how long to get to 1
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1 (initial
vote)
1 (nal vote)
2 (initial
vote)
2 (nal vote)
3 (initial
vote)
Question
Method
Sticker vote
50
25
60
48
Hand vote
4 weeks
When improvement criteria are rst met
20
4
Sticker vote
43
Hand vote
Sticker vote
Sticker vote
3 (nal vote)
Hand vote
No voting
5
6
7 (initial
vote)
7 (nal vote)
Votes
3 (second
vote)
4 (initial
vote)
Responses
Sticker vote
Hand vote
Sticker vote
Hand vote
23
25
24
20
66
29
28
24
3
56
38
15
NA
NA
19
1
1
45
38
14
3
* NA not applicable.
Only responses with substantial support are included.
550
Huber et al
DISCUSSION
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 rst
consensus meeting (4), the resulting 3 complete treatment
plans describe typical therapy for children with moderate
551
0 weeks (100%)
4 weeks
6 weeks
8 weeks (75%)
10 weeks
12 weeks
14 weeks (50%)
16 weeks
18 weeks
20 weeks
22 weeks (25%)
24 weeks
26 weeks
28 weeks
30 weeks
32 weeks
34 weeks
36 weeks (10%)
38 weeks
40 weeks
42 weeks
44 weeks
46 weeks
50 weeks (0%)
Any weight
10 kg
15 kg
20 kg
25 kg
>30 kg
2 mg/kg
20
17.5
1.5 mg/kg
15
12.5
1.0 mg/kg
10
30
27.5
25
22.5
20
17.5
15
12.5
10
40
35
32.5
30
25
22.5
20
17.5
15
12.5
10
8
50
45
40
37.5
32.5
27.5
25
20
17.5
15
12.5
10
7.5
60
55
50
45
40
35
30
25
20
17.5
15
12.5
10
7.5
7.5
0.5 mg/kg
7.5
5
6
5
2.5
1.5
2
0.2 mg/kg
2.5
1
1
2
1
1
Off
1
0
* 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.
552
Huber et al
Figure 1. Patient ow diagram for consensus treatment plans. JDM juvenile dermatomyositis; IVIG intravenous immunoglobulin.
ACKNOWLEDGMENTS
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.
AUTHOR CONTRIBUTIONS
All authors were involved in drafting the article or revising it
critically for important intellectual content, and all authors ap-
proved the nal 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, BoutTabaku, Carrasco, Curran, Feldman, Gewanter, Grifn, Haines,
Hoeltzel, Isgro, Kahn, Lang, Lawler, Shaham, Schmeling, Scuccimarri, Shishov, Stringer, Wohrley, Ilowite, Wallace.
Analysis and interpretation of data. Huber, Wohrley, Wallace.
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