Developing Best Practices for JDM for International Use

We are asking you to take part in a survey study being done through Susan Kim at the University of California, San Francisco. Participation is optional. Choosing to participate or choosing to not participate will not have any impact on participant's professional standing.

If you choose to participate, you will complete a survey. This survey will help us learn more about the practice patterns in Juvenile Dermatomyositis, and the use of the SHARE recommendations across the world. The survey will take about 10-15 minutes to complete.

You can skip questions that you do not want to answer or stop the survey at any time. The survey is anonymous, and no one will be able to link your answers back to you. Please do not include your name or other information that could be used to identify you in the survey responses.

Questions? Please contact Susan Kim at susan.kim@ucsf.edu. If you have questions or concerns about your rights as a research participant, you can call the UCSF Institutional Review Board at 415-476-1814.

If you want to participate in this survey, click the "Accept" button to start the survey.

Do you diagnose and treat patients with Juvenile Dermatomyositis (JDM)?

What is your role in caring for a patient with JDM?

How long have you been treating patients with JDM ?

Approximately how many patients per year do you care for with JDM?

Were you previously aware of the SHARE recommendations for JDM?

Have you used the SHARE recommendations in practice (either for yourself / your trainees)?

Do you think the SHARE recommendations need updating?

If yes, please list items you think need updating here:

In what country do you provide care for patients?

In what setting(s) do you care for most of your patients with JDM?

All children with suspected idiopathic inflammatory myopathies should be referred to a specialised centre.

High-risk patients need immediate/urgent referral to a specialised centre.

High risk defined by:
*Severe disability, defined by inability to get off
bed
*CMAS score < 15, or MMT8 score < 30
*Presence of aspiration or dysphagia (to the point of inability to swallow)
*Gastrointestinal vasculitis (as determined by
imaging or presence of bloody stools)
*Myocarditis
*Parenchymal lung disease
*Central nervous system disease (defined as decreased level of consciousness or seizures)
*Skin ulceration
*Requirement for intensive care unit management *Age < 1 year.     

Link of Measures and Definitions 

For JDM, patient-/parent-reported outcome measures are helpful when assessing disease activity and should be used at diagnosis and during disease monitoring.

Validated tools should be used to measure health status, for example, the Childhood Health Assessment Questionnaire, patient/parent visual analogue scale, Childhood Health Questionnaire, Juvenile Dermatomyositis Multi-dimensional Assessment Report.

All children with JDM should have disease activity (muscle, skin, major organ) assessed regularly in a standardised way, using tools such as the Disease Activity Score.

All children with JDM should have disease damage assessed at least yearly using a standardised disease damage measure, such as the Myositis Damage Index.

All patients with JDM should have the opportunity to be registered within a research registry/repository, for example, the Euromyositis registry.

Are there alternative investigations that you use that are not listed above? Please list / add comments here:

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Are there alternative assessments of muscle involvement that you use that are not listed above? Please list these here.

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Are there alternative assessments of skin involvement that you use that are not listed above? Please list these here.

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Are there alternative assessments of lung involvement that you use that are not listed above?  Please list these here:

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Are there alternative assessments of cardiac involvement that you use that are not listed above?  Please list these here.

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Are there alternative assessments of Calcinosis involvement that you use that are not listed above?  Please list these here.

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Are there alternative investigations that you use that are not listed above? Please list / add comments here:

Sun protection, including the routine use of sunblock on sun-exposed areas should be encouraged for patients with JDM.

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When treating patients with JDM, it is particularly important to have a physiotherapist and a specialist nurse actively involved as part of a multidisciplinary team.

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Treatment of JDM should include a safe and appropriate exercise programme, monitored by a physiotherapist.

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We recommend the induction regimen for treatment of new onset patients with JDM to be based on high dose of corticosteroids (oral or intravenous) combined with methotrexate.

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High-dose corticosteroids should be administered systemically either orally or intravenously in moderate-severe JDM.

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High-dose corticosteroids should be administered intravenously if there are concerns about absorption.

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Corticosteroid dose should be weaned as the patient shows clinical improvement.

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Addition of methotrexate or ciclosporin A leads to better disease control than prednisolone alone; safety profiles favour the combination of methotrexate and prednisolone.

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Methotrexate should be started at a dose of 15-20 mg/m2/week (max absolute dose of 40 mg /week) preferably administered subcutaneously at disease onset.

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If a newly diagnosed patient has inadequate response to treatment, intensification of treatment should be considered within the first 12 weeks, after consultation with an expert centre.

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Intravenous immunoglobulin may be a useful adjunct for resistant disease, particularly when skin features are prominent.

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MMF (mycophenolate mofetil) may be a useful therapy for muscle and skin disease (including calcinosis).

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Ongoing skin disease reflects ongoing systemic disease and therefore should be treated by increasing systemic immunosuppression. Topical tacrolimus (0.1%)/topical steroids may help localised skin disease, particularly for symptomatic redness or itching.

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Are there other treatment recommendations you commonly make not noted above? Please list these here.

THANK YOU FOR TAKING THIS SURVEY!

Please leave additional questions or comments here.

Please consider being more involved with this work and email us so you can be included in future discussions and meetings : Susan.kim@ucsf.edu