Background/Purpose: At present no clear evidence based guidelines exist to standardize the tapering and discontinuation of corticosteroids (CS) in juvenile dermatomyositis (JDM). To provide evidence-based recommendations for CS tapering/discontinuation through the analysis of the patients in the PRINTO new onset JDM trial and to identify predictors of clinical remission and CS discontinuation.

Methods: New onset JDM children were randomized to receive either prednisone (PDN) alone or in combination with MTX or CSA, according to a specific steroid protocol. Major therapeutic changes (MTC) were defined as the addition or major increase in the dose of MTX/CSA/other drugs or any other reasons for which patients were dropped from the trial. Patients were divided according to clinical remission into two major groups. Group 1 included those on clinical remission, who could discontinue PDN, with no MTC, and represented the reference standard for the best clinical outcome. Group 1 was compared with those who did not achieve clinical remission, without or with MTC (group 2 and 3, respectively). JDM core set measures (CSM) were compared in the 3 groups. We also calculated the gold standard group (group 1) median change in the CSM in the first 6 and over 24 months and applied a logistic regression model to identify the predictors of clinical remission with PDN discontinuation.

Results: 139 children were enrolled in the trial: 47 on PDN, 46 on PDN+CSA and 46 on PDN+MTX. We identified 30 (21.6%) patients for group 1, 43 (30.9%) for group 2 and 66 (47.5%) for group 3. At baseline all 3 groups had a high level of disease activity with no differences in the CSM. Already in the first 2 months a clear differential trend in disease activity measures, according to clinical remission status and PDN discontinuation, could be identified. From the observation of the median change in the CSM of group 1 in the first 6 months, the following recommendations could be extrapolated: decrease corticosteroids from 2 to 1 mg/kg/day in 2 months if the MD-global, parent-global, CHAQ, DAS, CMAS, MMT or Phs measures have a change of at least 50%; from 1 to 0.5 mg/kg/day in the following 2 months if the MD-global, CHAQ, DAS, CMAS have a change of at least 20%; in the following 2 months (month 4-6) corticosteroids can be tapered up to the safe dose of 0.2 mg/kg/day, if the disease activity measures remain at low/normal values. We finally ran a logistic regression model that showed that the achievement of PRINTO criteria 50-70-90 at 2 months from disease onset, an age at onset >9 years and the combination therapy PDN+MTX, increase the probability of clinical remission from 4 to 7 times. (table 1)

Conclusion: We propose evidence based specific cut-offs for CS tapering/discontinuation based on the change in JDM CSM of disease activity, and identify the best predictors for clinical remission.

Table 1. Logistic regression model for the outcome remission

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/evidence-based-recommendations-for-corticosteroid-taperingdiscontinuation-in-new-onset-juvenile-dermatomyositis-patients-from-the-printo-trial/