The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients

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dc.contributor.authorGiancane, Gabriella
dc.contributor.authorLavarello, Claudio
dc.contributor.authorPistorio, Angela
dc.contributor.authorOliveira, Sheila K.
dc.contributor.authorZulian, Francesco
dc.contributor.authorCuttica, Ruben
dc.contributor.authorFischbach, Michel
dc.contributor.authorMagnusson, Bo
dc.contributor.authorPastore, Serena
dc.contributor.authorMarini, Roberto
dc.contributor.authorMartino, Silvana
dc.contributor.authorPagnier, Anne
dc.contributor.authorSoler, Christine
dc.contributor.authorStanevicha, Valda
dc.contributor.authorTen Cate, Rebecca
dc.contributor.authorUziel, Yosef
dc.contributor.authorVojinovic, Jelena
dc.contributor.authorFueri, Elena
dc.contributor.authorRavelli, Angelo
dc.contributor.authorMartini, Alberto
dc.contributor.authorRuperto, Nicolino
dc.date.accessioned2021-05-31T07:35:01Z
dc.date.available2021-05-31T07:35:01Z
dc.date.issued2019-05-22
dc.description.abstractBackgroundPrednisone (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.en
dc.description.statusPeer reviewed
dc.description.versionpublishersversion
dc.identifier.citationGiancane , G , Lavarello , C , Pistorio , A , Oliveira , S K , Zulian , F , Cuttica , R , Fischbach , M , Magnusson , B , Pastore , S , Marini , R , Martino , S , Pagnier , A , Soler , C , Stanevicha , V , Ten Cate , R , Uziel , Y , Vojinovic , J , Fueri , E , Ravelli , A , Martini , A & Ruperto , N 2019 , ' The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients ' , Pediatric Rheumatology , vol. 17 , 24 . https://doi.org/10.1186/s12969-019-0326-5
dc.identifier.doihttps://doi.org/10.1186/s12969-019-0326-5
dc.identifier.issn1546-0096
dc.identifier.otherPURE: 25626927
dc.identifier.otherPURE UUID: 98139da5-0562-4e2c-a4d3-d90eacbbdb6c
dc.identifier.otherWOS: 000468851200001
dc.identifier.urihttps://dspace.rsu.lv/jspui/handle/123456789/4576
dc.identifier.urlhttps://ped-rheum.biomedcentral.com/articles/10.1186/s12969-019-0326-5
dc.language.isoeng
dc.relation.ispartofPediatric Rheumatology
dc.rights
dc.subjectJuvenile dermatomyositis
dc.subjectPrednisone tapering
dc.subjectGlucorticoids
dc.subjectDisease activity
dc.subjectCore set measures
dc.subject3.2 Clinical medicine
dc.subject1.1. Scientific article indexed in Web of Science and/or Scopus database
dc.titleThe PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patientsen
dc.type/dk/atira/pure/researchoutput/researchoutputtypes/contributiontojournal/article

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