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Research article
Anthony R. French
Washington University School of Medicine
Corresponding Author
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Background
High-intensity glucocorticoid regimens are commonly used to induce and maintain remission in Juvenile Dermatomyositis (JDM) but are associated with several adverse side-effects. Eminence-based treatment guidelines from CARRA and SHARE both advocate induction of intravenous pulse steroids followed by high dose oral steroids (2 mg/kg) tapered over 12 months. This study compares the time to disease control with reduced glucocorticoid dosing.
Methods
We retrospectively reviewed the records at a single tertiary-care children’s hospital of patients with JDM between 2000 and 2014 who had a minimum of 2 years of follow-up. The primary outcome measure was time to control of muscle and skin disease. Additional outcome measures included glucocorticoid dosing, effect of treatment on height, frequency of calcinosis and complications from treatment.
Results
Of the 69 patients followed during the study period, 31 fulfilled inclusion criteria. Median length of follow-up was 4.58 years, (3–7.5). Myositis control was achieved in a median of 7.1 months (range 0.9–63.4). Cutaneous disease control was achieved in a median of 16.7 months (range 4.3–89.5). The median starting dose of glucocorticoids was 0.85 mg/kg/day, (range 0.5–1.74). The median duration of steroid treatment was 9.1 months, (range 4.7–17.4), while the median duration of any pharmacotherapy was 29.2 months (range 10.4 to 121.3). Sustained disease control off medications was achieved in 21/31 (68%) patients by the end of review. Persistent calcinosis was identified in only one patient (3%).
Conclusion
Current accepted treatment paradigms for JDM include oral glucocorticoids beginning at 2 mg/kg/d reduced over 12 months; however, our results suggest that treatment using reduced doses and durations with early use of steroid-sparing agents are comparably effective in achieving favorable outcomes in JDM.
Keywords
Juvenile Dermatomyositis, Calcinosis, Biologic Therapy, Glucocorticoids, Pediatric Rheumatology
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CURRENT STATUS: Published
View the published article at Pediatric Rheumatology.
Version (no preprint)
Posted 11 Feb, 2021
Reviews received
Received 11 Feb, 2021
Reviewers invited
Invitations sent on 09 Feb, 2021
First submitted
On 04 Jan, 2021
This version was not preprinted
Version 1
Posted 13 Jan, 2021
No community comments so far
Editorial decision: Major revision
On 04 Apr, 2021
Review #9 received
Received 24 Feb, 2021
Reviewer #9 agreed
On 11 Feb, 2021
Review #8 received
Received 11 Feb, 2021
Review #5 received
Received 09 Feb, 2021
Reviewer #7 agreed
On 27 Jan, 2021
Reviewer #8 agreed
On 27 Jan, 2021
Reviewer #6 agreed
On 26 Jan, 2021
Review #6 received
Received 26 Jan, 2021
Review #7 received
Received 26 Jan, 2021
Reviewer #3 agreed
On 23 Jan, 2021
Reviewer #4 agreed
On 23 Jan, 2021
Reviewer #5 agreed
On 23 Jan, 2021
Review #3 received
Received 23 Jan, 2021
Review #4 received
Received 23 Jan, 2021
Reviewer #2 agreed
On 23 Jan, 2021
Review #2 received
Received 23 Jan, 2021
Review #1 received
Received 11 Jan, 2021
Reviewer #1 agreed
On 09 Jan, 2021
Reviewers invited
Invitations sent on 06 Jan, 2021
Editor assigned
On 04 Jan, 2021
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On 04 Jan, 2021
Editor invited
On 04 Jan, 2021
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See the published version of this preprint at Pediatric Rheumatology.
This is a preprint, a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research article
Anthony R. French
Washington University School of Medicine
Corresponding Author
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Published
View the published article at Pediatric Rheumatology.
Version (no preprint)
Posted 11 Feb, 2021
Reviews received
Received 11 Feb, 2021
Reviewers invited
Invitations sent on 09 Feb, 2021
First submitted
On 04 Jan, 2021
This version was not preprinted
Version 1
Posted 13 Jan, 2021
No community comments so far
Editorial decision: Major revision
On 04 Apr, 2021
Review #9 received
Received 24 Feb, 2021
Reviewer #9 agreed
On 11 Feb, 2021
Review #8 received
Received 11 Feb, 2021
Review #5 received
Received 09 Feb, 2021
Reviewer #7 agreed
On 27 Jan, 2021
Reviewer #8 agreed
On 27 Jan, 2021
Reviewer #6 agreed
On 26 Jan, 2021
Review #6 received
Received 26 Jan, 2021
Review #7 received
Received 26 Jan, 2021
Reviewer #3 agreed
On 23 Jan, 2021
Reviewer #4 agreed
On 23 Jan, 2021
Reviewer #5 agreed
On 23 Jan, 2021
Review #3 received
Received 23 Jan, 2021
Review #4 received
Received 23 Jan, 2021
Reviewer #2 agreed
On 23 Jan, 2021
Review #2 received
Received 23 Jan, 2021
Review #1 received
Received 11 Jan, 2021
Reviewer #1 agreed
On 09 Jan, 2021
Reviewers invited
Invitations sent on 06 Jan, 2021
Editor assigned
On 04 Jan, 2021
Submission checks complete
On 04 Jan, 2021
Editor invited
On 04 Jan, 2021
First submitted
On 03 Jan, 2021
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
License:
This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at Pediatric Rheumatology.
Background
High-intensity glucocorticoid regimens are commonly used to induce and maintain remission in Juvenile Dermatomyositis (JDM) but are associated with several adverse side-effects. Eminence-based treatment guidelines from CARRA and SHARE both advocate induction of intravenous pulse steroids followed by high dose oral steroids (2 mg/kg) tapered over 12 months. This study compares the time to disease control with reduced glucocorticoid dosing.
Methods
We retrospectively reviewed the records at a single tertiary-care children’s hospital of patients with JDM between 2000 and 2014 who had a minimum of 2 years of follow-up. The primary outcome measure was time to control of muscle and skin disease. Additional outcome measures included glucocorticoid dosing, effect of treatment on height, frequency of calcinosis and complications from treatment.
Results
Of the 69 patients followed during the study period, 31 fulfilled inclusion criteria. Median length of follow-up was 4.58 years, (3–7.5). Myositis control was achieved in a median of 7.1 months (range 0.9–63.4). Cutaneous disease control was achieved in a median of 16.7 months (range 4.3–89.5). The median starting dose of glucocorticoids was 0.85 mg/kg/day, (range 0.5–1.74). The median duration of steroid treatment was 9.1 months, (range 4.7–17.4), while the median duration of any pharmacotherapy was 29.2 months (range 10.4 to 121.3). Sustained disease control off medications was achieved in 21/31 (68%) patients by the end of review. Persistent calcinosis was identified in only one patient (3%).
Conclusion
Current accepted treatment paradigms for JDM include oral glucocorticoids beginning at 2 mg/kg/d reduced over 12 months; however, our results suggest that treatment using reduced doses and durations with early use of steroid-sparing agents are comparably effective in achieving favorable outcomes in JDM.
Figure 1
Figure 2
Figure 3
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