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

Gabriella Giancane; Claudio Lavarello; Angela Pistorio; Sheila K. Oliveira; Francesco Zulian; Ruben Cuttica; Michel Fischbach; Bo Magnusson; Serena Pastore; Roberto Marini; Silvana Martino; Anne Pagnier; Christine Soler; Valda Stanevicha; Rebecca Ten Cate; Yosef Uziel; Jelena Vojinovic; Elena Fueri; Angelo Ravelli; Alberto Martini; Nicolino Ruperto

doi:10.1186/s12969-019-0326-5

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

Gabriella Giancane, Claudio Lavarello, Angela Pistorio, Sheila K. Oliveira, Francesco Zulian, Ruben Cuttica, Michel Fischbach, Bo Magnusson, Serena Pastore, Roberto Marini, Silvana Martino, Anne Pagnier, Christine Soler, Valda Stanevicha, Rebecca Ten Cate, Yosef Uziel, Jelena Vojinovic, Elena Fueri, Angelo Ravelli, Alberto MartiniNicolino Ruperto

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Abstract

BackgroundPrednisone (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.

Original language	English
Article number	24
Journal	Pediatric Rheumatology
Volume	17
DOIs	https://doi.org/10.1186/s12969-019-0326-5
Publication status	Published - 22 May 2019
Externally published	Yes

Keywords*

Juvenile dermatomyositis
Prednisone tapering
Glucorticoids
Disease activity
Core set measures

Field of Science*

3.2 Clinical medicine

Publication Type*

1.1. Scientific article indexed in Web of Science and/or Scopus database

Access to Document

10.1186/s12969-019-0326-5Licence: CC BY

The PRINTO evidence-based proposal forFinal published version, 1.37 MBLicence: CC BY

https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-019-0326-5Licence: CC BY

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Giancane, 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., ... Ruperto, N. (2019). The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients. Pediatric Rheumatology, 17, Article 24. https://doi.org/10.1186/s12969-019-0326-5

@article{98139da505624e2ca4d3d90eacbbdb6c,

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

abstract = "BackgroundPrednisone (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.",

keywords = "Juvenile dermatomyositis, Prednisone tapering, Glucorticoids, Disease activity, Core set measures",

author = "Gabriella Giancane and Claudio Lavarello and Angela Pistorio and Oliveira, {Sheila K.} and Francesco Zulian and Ruben Cuttica and Michel Fischbach and Bo Magnusson and Serena Pastore and Roberto Marini and Silvana Martino and Anne Pagnier and Christine Soler and Valda Stanevicha and {Ten Cate}, Rebecca and Yosef Uziel and Jelena Vojinovic and Elena Fueri and Angelo Ravelli and Alberto Martini and Nicolino Ruperto",

year = "2019",

month = may,

day = "22",

doi = "10.1186/s12969-019-0326-5",

language = "English",

volume = "17",

journal = "Pediatric Rheumatology",

issn = "1546-0096",

publisher = "BioMed Central Ltd.",

}

Giancane, G, Lavarello, C, Pistorio, A, Oliveira, SK, 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

TY - JOUR

T1 - The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients

AU - Giancane, Gabriella

AU - Lavarello, Claudio

AU - Pistorio, Angela

AU - Oliveira, Sheila K.

AU - Zulian, Francesco

AU - Cuttica, Ruben

AU - Fischbach, Michel

AU - Magnusson, Bo

AU - Pastore, Serena

AU - Marini, Roberto

AU - Martino, Silvana

AU - Pagnier, Anne

AU - Soler, Christine

AU - Stanevicha, Valda

AU - Ten Cate, Rebecca

AU - Uziel, Yosef

AU - Vojinovic, Jelena

AU - Fueri, Elena

AU - Ravelli, Angelo

AU - Martini, Alberto

AU - Ruperto, Nicolino

PY - 2019/5/22

Y1 - 2019/5/22

N2 - BackgroundPrednisone (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.

AB - BackgroundPrednisone (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.

KW - Juvenile dermatomyositis

KW - Prednisone tapering

KW - Glucorticoids

KW - Disease activity

KW - Core set measures

U2 - 10.1186/s12969-019-0326-5

DO - 10.1186/s12969-019-0326-5

M3 - Article

SN - 1546-0096

VL - 17

JO - Pediatric Rheumatology

JF - Pediatric Rheumatology

M1 - 24

ER -

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

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