Please use this identifier to cite or link to this item:
Type: Artigo
Title: The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients
Author: Giancane, Gabriella
Lavarello, Claudio
Pistorio, Angela
Oliveira, Sheila K.
Zulian, Francesco
Cuttica, Ruben
Fischbach, Michel
Magnusson, Bo
Pastore, Serena
Marini, Roberto
Martino, Silvana
Pagnier, Anne
Soler, Christine
Stanevicha, Valda
Ten Cate, Rebecca
Uziel, Yosef
Vojinovic, Jelena
Fueri, Elena
Ravelli, Angelo
Martini, Alberto
Ruperto, Nicolino
Abstract: Background: Prednisone (PDN) in juvenile dermatomyositis (JDM), alone or in association with other immunosuppressive drugs, namely methotrexate (MTX) and cyclosporine (CSA), represents the first-line treatment option for new onset JDM patients. No clear evidence based guidelines are actually available to standardize the tapering and discontinuation of glucocorticoids (GC) in JDM. Aim of our study was to provide an evidence-based proposal for GC tapering/discontinuation in new onset juvenile dermatomyositis (JDM), and to identify predictors of clinical remission and GC discontinuation.MethodsNew onset JDM children were randomized to receive either PDN alone or in combination with methotrexate (MTX) or cyclosporine (CSA). In order to derive steroid tapering indications, PRINTO/ACR/EULAR JDM core set measures (CSM) and their median absolute and relative percent changes over time were compared in 3 groups. Group 1 included those in clinical remission who discontinued PDN, with no major therapeutic changes (MTC) (reference group) and was compared with those who did not achieve clinical remission, without or with MTC (Group 2 and 3, respectively). A logistic regression model identified predictors of clinical remission with PDN discontinuation.ResultsBased on the median change in the CSM of 30/139 children in Group 1, after 3 pulses of methyl-prednisolone, GC could be tapered from 2 to 1mg/kg/day in the first two months from onset if any of the CSM decreased by 50-94%, and from 1 to 0.2mg/kg/day in the following 4months if any CSM further decreased by 8-68%, followed by discontinuation in the ensuing 18months. The achievement of PRINTO JDM 50-70-90 response after 2months of treatment (ORs range 4.5-6.9), an age at onset >9years (OR 4.6) and the combination therapy PDN+MTX (OR 3.6) increase the probability of achieving clinical remission (p<0.05).ConclusionsThis is the first evidence-based proposal for glucocorticoid tapering/discontinuation based on the change in JDM CSM of disease activity.
Subject: Dermatomiosite
Country: Reino Unido
Editor: BioMed
Rights: Aberto
Identifier DOI: 10.1186/s12969-019-0326-5
Date Issue: 2019
Appears in Collections:FCM - Artigos e Outros Documentos

Files in This Item:
File Description SizeFormat 
000468851200001.pdf1.45 MBAdobe PDFView/Open

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.