Practice Patterns for Amyopathic Juvenile Dermatomyositis

The Childhood Arthritis and Rheumatology Research Alliance (CARRA) is a North American organization almost 400 pediatric rheumatologists & researchers who are working together to find better ways to treat pediatric rheumatologic conditions (https://www.carragroup.org).

As you know, there are no “proven” treatments for JDM due to the rarity of the condition, as well as the difficulty funding large clinical trials. CARRA has developed ‘Consensus Treatment Plans’ for Moderate JDM which is currently in its pilot phase (see links below). Our intent is to allow clinicians to choose the treatment plan that is similar to their usual practice. By doing so, variation will be reduced, and large numbers of children will be treated similarly. This will allow for prospective data collection and application of newer statistical methods (eg: comparative effectiveness, propensity scoring) to help determine the most effective therapy.

Similarly, we would like to try to develop ‘Consensus Treatment Plans’ for "amyopathic" JDM. To develop representative treatment plans for "amyopathic" JDM, in addition to the Rheumatology CARRA community, we need to engage the Pediatric Dermatology Community and are hoping to collaborate with the SPD. Sheilagh Maguiness (Pediatric Dermatology of SPD) has helped to coordinate this effort.

This survey will help us understand the degree of overlap in the evaluation and treatment approaches for children with JDM between CARRA and SPD, and will be the first step to determine if a future collaboration with the CARRA JDM group and SPD could be successful.

This is an IRB approved survey. Proceeding with this web-based survey will imply your consent to participate in this study, and participants will remain anonymous. If this work is fruitful, our intention would be continued collaboration with SPD and possible publication in the future.

Thank you in advance for your help with this rare condition!
Susan Kim (Pediatric Rheumatology)

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2909837/
http://onlinelibrary.wiley.com/doi/10.1002/acr.20695/pdf

Question Title

* 1. How long have you been practicing pediatric dermatology?

0-2 years

3-5 years

6-9 years

>10 years

Other (please specify)

Question Title

* 2. In what setting do you practice pediatric dermatology?

Academic Medical Center

Private Medical Center

Private Practice

Industry

Retired

If more than one setting, please estimate breakdown of time:

Question Title

* 3. Please estimate how many patients with juvenile dermatomyositis you have seen in the past 5 years:

none

1-5

6-10

11-15

16-20

>20

Other (please specify)

Question Title

* 4. Please estimate how many patients with juvenile dermatomyositis you actively follow?

none

1-5

6-10

11-15

16-20

>20

Other (please specify)

Question Title

* 5. Please estimate how many patients with Amyopathic juvenile dermatomyositis have you seen in the past 5 years?

none

1-5

6-10

11-15

16-20

>20

If you can estimate a # please list here:

Question Title

* 6. Please estimate how many patients with Amyopathic juvenile dermatomyositis you actively follow?

none

1-5

6-10

11-15

16-20

>20

If you can estimate a # please list here:

Question Title

* 7. In your opinion, how often do patients with Amyopathic juvenile dermatomyositis ultimately develop muscle disease?

never

<25%

25-50%

>50%-75%

>75%

always

Other

Question Title

* 8. Do you screen for muscle disease?

Not applicable

never

initially

all visits

Other

Question Title

* 9. If you screen for muscle weakness, how do you screen for muscle weakness (choose all those that apply)?

	Not applicable	Never	Initially	All viisits
Ask the patient/parent if they are weak?	Ask the patient/parent if they are weak? Not applicable	Ask the patient/parent if they are weak? Never	Ask the patient/parent if they are weak? Initially	Ask the patient/parent if they are weak? All viisits
Perform formal strength testing for muscle weakness?	Perform formal strength testing for muscle weakness? Not applicable	Perform formal strength testing for muscle weakness? Never	Perform formal strength testing for muscle weakness? Initially	Perform formal strength testing for muscle weakness? All viisits
Perform laboratory testing?	Perform laboratory testing? Not applicable	Perform laboratory testing? Never	Perform laboratory testing? Initially	Perform laboratory testing? All viisits

Other Comments

Question Title

* 10. If you perform lab testing, which muscle enzymes do you check, if any (check all those that apply)?

Not applicable

LDH

AST

ALT

Aldolase

Factor 8 related antigen

CBC

C-reactive protein

ESR

ANA

Myositis specific antigens

Myositis associated antigens

Other Lab Testing:

Question Title

* 11. Which additional testing do you perform?

	Not Applicable	Never	Sometimes	Always
EMG	EMG Not Applicable	EMG Never	EMG Sometimes	EMG Always
MRI	MRI Not Applicable	MRI Never	MRI Sometimes	MRI Always
Skin biopsy	Skin biopsy Not Applicable	Skin biopsy Never	Skin biopsy Sometimes	Skin biopsy Always
Muscle biopsy	Muscle biopsy Not Applicable	Muscle biopsy Never	Muscle biopsy Sometimes	Muscle biopsy Always

Other Testing

Question Title

* 12. How often do you perform skin biopsies to confirm juvenile dermatomyositis?

Not applicable

never

<25%

25-50%

>50%, <75%

always

Other (please specify)

Question Title

* 13. How often do you refer patients with juvenile dermatomyositis to a pediatric rheumatologist?

never

<25%

25-50%

>50%, <75%

>75%

always

Other (please specify)

Question Title

* 14. If you do not refer patients with juvenile dermatomyositis to a pediatric rheumatologist, why not (choose all that apply)?

No availability of pediatric rheumatologist

No clinical need for pediatric rheumatologist

Other (please specify)

Question Title

* 15. How often do you refer patients with Amyopathic juvenile dermatomyositis to a pediatric rheumatologist?

Not applicable

never

<25%

25-50%

>50%, <75%

>75%

always

Other (please specify)

Question Title

* 16. In addition to sunscreen/protection, please rank the topical therapies you would use for dermatomyositis:

	1	2	3	4	5
Low potency steroids	Low potency steroids 1	Low potency steroids 2	Low potency steroids 3	Low potency steroids 4	Low potency steroids 5
Mid potency steroids	Mid potency steroids 1	Mid potency steroids 2	Mid potency steroids 3	Mid potency steroids 4	Mid potency steroids 5
High potency steroids	High potency steroids 1	High potency steroids 2	High potency steroids 3	High potency steroids 4	High potency steroids 5
Calineurin inhibitors	Calineurin inhibitors 1	Calineurin inhibitors 2	Calineurin inhibitors 3	Calineurin inhibitors 4	Calineurin inhibitors 5
Other	Other 1	Other 2	Other 3	Other 4	Other 5

Other (please specify)

Question Title

* 17. Are there any guidelines you follow regarding topical therapy?

Yes

If yes, please specify

Question Title

* 18. When would you consider the addition of a systemic agent for Amyopathic dermatomyositis (check all those that apply)?

Location of rash

Extent of rash

Duration of rash

Ulceration

Calcinosis

Development of muscle weakness

Development of systemic symptoms

If you checked "development of systemic symptoms" please list specific symptoms that you consider

Question Title

* 19. What systemic therapies do you use/would you use, for your patients with juvenile dermatomyositis?

none

hydroxychloroquine

chloroquine

methotrexate

cyclosporine

tacrolimus

mycophenolate mofetil

oral steroids

intravenous steroids

IV immunoglobulin

Other therapy:

Question Title

* 20. Do you think that better treatment and understanding of Amyopathic JDM is important?

Yes

Question Title

* 21. Would you be interested in collaborating to work to better treat and understand Amyopathic JDM in the future?

Yes

If yes, please provide email address/contact information in a separate email to: susan.kim@childrens.harvard.edu

Question Title

* 1. How long have you been practicing pediatric dermatology?

Question Title

* 2. In what setting do you practice pediatric dermatology?

Question Title

* 3. Please estimate how many patients with juvenile dermatomyositis you have seen in the past 5 years:

Question Title

* 4. Please estimate how many patients with juvenile dermatomyositis you actively follow?

Question Title

* 5. Please estimate how many patients with Amyopathic juvenile dermatomyositis have you seen in the past 5 years?

Question Title

* 6. Please estimate how many patients with Amyopathic juvenile dermatomyositis you actively follow?

Question Title

* 7. In your opinion, how often do patients with Amyopathic juvenile dermatomyositis ultimately develop muscle disease?

Question Title

* 8. Do you screen for muscle disease?

Question Title

* 9. If you screen for muscle weakness, how do you screen for muscle weakness (choose all those that apply)?

Question Title

* 10. If you perform lab testing, which muscle enzymes do you check, if any (check all those that apply)?

Question Title

* 11. Which additional testing do you perform?

Question Title

* 12. How often do you perform skin biopsies to confirm juvenile dermatomyositis?

Question Title

* 13. How often do you refer patients with juvenile dermatomyositis to a pediatric rheumatologist?

Question Title

* 14. If you do not refer patients with juvenile dermatomyositis to a pediatric rheumatologist, why not (choose all that apply)?

Question Title

* 15. How often do you refer patients with Amyopathic juvenile dermatomyositis to a pediatric rheumatologist?

Question Title

* 16. In addition to sunscreen/protection, please rank the topical therapies you would use for dermatomyositis:

Question Title

* 17. Are there any guidelines you follow regarding topical therapy?

Question Title

* 18. When would you consider the addition of a systemic agent for Amyopathic dermatomyositis (check all those that apply)?

Question Title

* 19. What systemic therapies do you use/would you use, for your patients with juvenile dermatomyositis?

Question Title

* 20. Do you think that better treatment and understanding of Amyopathic JDM is important?

Question Title

* 21. Would you be interested in collaborating to work to better treat and understand Amyopathic JDM in the future?

Question Title

* 22. Do you have any other comments, questions, or concerns?