Evidence Based Recommendations for Corticosteroid Tapering/Discontinuation in New Onset Juvenile Dermatomyositis Patients: Results from the Paediatric Rheumatology International Trials Organisation

Gabriella Giancane¹, Claudio Lavarello², Angela Pistorio², Francesco Zulian², Bo Magnusson³, Tadej Avcin², Fabrizia Corona², Valeria Gerloni², Serena Pastore², Roberto Marini², Silvana Martino², Anne Pagnier⁴, Michel Rodiere², Christine Soler², Valda Stanevicha², Rebecca ten Cate², Yosef Uziel², Jelena Vojinovic², Elena Fueri², Angelo Ravelli⁵, Alberto Martini⁶ and Nicola Ruperto⁷, ¹Clinica Pediatrica - Reumatologia, Istituto Giannina Gaslini, Genoa, Italy, ²Istituto Giannina Gaslini - Clinica Pediatrica e Reumatologia - PRINTO, Genoa, Italy, ³Karolinska University Hospital, Stockholm, Sweden, ⁴Istituto Giannina Gaslini - Clinica Pediatrica e Reumatologia - PRINTO, Genova, Italy, ⁵Clinica Pediatrica - Reumatologia, Istituto Giannina Gaslini, Genova, Italy, ⁶Universita di Genova Pediatria II, Genova, Italy, ⁷Istituto Giannina Gaslini - Clinica Pediatrica e Reumatologia, Genoa, Italy

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: clinical trials, corticosteroids, juvenile dermatomyositis and remission

Session Information

Date: Sunday, October 21, 2018

Title: Pediatric Rheumatology – Clinical Poster I: Lupus, Sjögren’s Disease, and Myositis

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: At present no clear evidence based guidelines exist to standardize the tapering and discontinuation of corticosteroids (CS) in juvenile dermatomyositis (JDM). Aim of our study is 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 (CR) and CS discontinuation.

Methods: New onset JDM children were randomized to receive either prednisone (PDN) alone or in combination with methotrexate (MTX) or cyclosporine (CSA). We stratified patients according to CR into two major groups. Group 1 included those on CR, who could discontinue PDN, with no major therapeutic changes (MTC) (reference group). Group 1 was compared with those who did not achieve CR, without or with MTC (group 2 and 3, respectively). PRINTO/ACR/EULAR JDM core set measures (CSM) and their median changes over time were compared in the 3 groups. A logistic regression model with odds ratios (OR) and 95% confidence intervals (CI) was used to identify predictors of CR with PDN discontinuation.

Results: 139 children were enrolled. The reference Group 1 showed at least a 50% decrease in the CSM already in the first 2 months when compared to the other two groups. The achievement of a PRINTO JDM 50-70-90 response at 2 months (ORs range 4.5-6.9) from treatment start, an age at onset >9 years (OR 4.6) and the combination therapy PDN+MTX (OR 3.6) increase the probability of achieving CR (p<0.05).(Table)

Conclusion: This is the first proposal of evidence based specific cut-offs for corticosteroid tapering/discontinuation based on the change in JDM CSM of disease activity.

Table	OR (95% CI)	P^#
Responder at 2 months: Printo-50 (vs. not responder/Printo-20) Printo-70 (vs. not responder/Printo-20) Printo-90 (vs. not responder/Printo-20) Age at onset > 8.53 years (£ 8.53 years)	5.41 (1.37 – 21.32) 6.90 (1.91 – 24.99) 4.46 (1.08 – 18.38) 4.64 (1.69 – 12.71)	0.0076 0.0017
Treatment group: PDN+MTX (vs. PDN / PDN+CSA)	3.63 (1.30 – 10.09)	0.0116
AUC of the model: 0.80

OR: Odds Ratio; 95% CI: 95% Confidence Interval; P^#: Likelihood Ratio test

Disclosure: G. Giancane, None; C. Lavarello, None; A. Pistorio, None; F. Zulian, None; B. Magnusson, None; T. Avcin, None; F. Corona, None; V. Gerloni, None; S. Pastore, None; R. Marini, None; S. Martino, None; A. Pagnier, None; M. Rodiere, None; C. Soler, None; V. Stanevicha, None; R. ten Cate, None; Y. Uziel, None; J. Vojinovic, None; E. Fueri, None; A. Ravelli, Abbvie, Bristol Myers Squibb, Novartis, Pfizer, Roche and Johnson & Johnson, 5, 8; A. Martini, None; N. Ruperto, Abbott, AbbVie, Amgen, Biogenidec, Astellas, Alter, AstraZeneca, Baxalta Biosimilars, Boehringer, BMS, CD-Pharma,Celgene, CrescendoBio, EMD Serono, Hoffman-La Roche, Italfarmaco, Janssen, MedImmune, Medac, Novartis, Novo Nordisk, Pfizer, Rewind Arms, R-Ph, 5, 8,Abbott, BMS, "Francesco Angelini", GlaxoSmithKline (GSK), Hoffman-La Roche, Italfarmaco, Janssen, Novartis, Pfizer, Sanofi Aventis, Schwarz Biosciences, Sobi, Xoma, Wyeth., 2.

To cite this abstract in AMA style:

Giancane G, Lavarello C, Pistorio A, Zulian F, Magnusson B, Avcin T, Corona F, Gerloni V, Pastore S, Marini R, Martino S, Pagnier A, Rodiere M, Soler C, Stanevicha V, ten Cate R, Uziel Y, Vojinovic J, Fueri E, Ravelli A, Martini A, Ruperto N. Evidence Based Recommendations for Corticosteroid Tapering/Discontinuation in New Onset Juvenile Dermatomyositis Patients: Results from the Paediatric Rheumatology International Trials Organisation [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/evidence-based-recommendations-for-corticosteroid-tapering-discontinuation-in-new-onset-juvenile-dermatomyositis-patients-results-from-the-paediatric-rheumatology-international-trials-organisation/. Accessed .

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