EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease

Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. Methods In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendatio...

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Autores: Fautrel B, Mitrovic S, De Matteis A, Bindoli S, Antón J, Belot A, Bracaglia C, Constantin T, Dagna L, Di Bartolo A, Feist E, Foell D, Gattorno M, Georgin-Lavialle S, Giacomelli R, Grom AA, Jamilloux Y, Laskari K, Lazar C, Minoia F, Nigrovic PA, Oliveira Ramos F, Ozen S, Quartier P, Ruscitti P, Sag E, Savic S, Truchetet ME, Vastert SJ, Wilhelmer TC, Wouters C, Carmona L, De Benedetti F
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p26900
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=26900
Access Level:acceso abierto
Palabra clave:Still's disease
adult-onset
arthritis
juvenile
macrophage activation syndrome
biological therapy
inflammation
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spelling EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's diseaseFautrel BMitrovic SDe Matteis ABindoli SAntón JBelot ABracaglia CConstantin TDagna LDi Bartolo AFeist EFoell DGattorno MGeorgin-Lavialle SGiacomelli RGrom AAJamilloux YLaskari KLazar CMinoia FNigrovic PAOliveira Ramos FOzen SQuartier PRuscitti PSag ESavic STruchetet MEVastert SJWilhelmer TCWouters CCarmona LDe Benedetti FStill's diseaseadult-onsetarthritisjuvenilemacrophage activation syndromebiological therapyinflammationSystemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. Methods In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly. Results The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still's disease. Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-gamma inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still's disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement. Conclusion These recommendations are the first consensus for the diagnosis and management of children and adults with Still's disease.ELSEVIER2024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=26900ANNALS OF THE RHEUMATIC DISEASESISSN: 00034967ISSNe: 14682060reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p269002026-05-27T12:37:41Z
dc.title.none.fl_str_mv EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
title EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
spellingShingle EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
Fautrel B
Still's disease
adult-onset
arthritis
juvenile
macrophage activation syndrome
biological therapy
inflammation
title_short EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
title_full EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
title_fullStr EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
title_full_unstemmed EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
title_sort EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease
dc.creator.none.fl_str_mv Fautrel B
Mitrovic S
De Matteis A
Bindoli S
Antón J
Belot A
Bracaglia C
Constantin T
Dagna L
Di Bartolo A
Feist E
Foell D
Gattorno M
Georgin-Lavialle S
Giacomelli R
Grom AA
Jamilloux Y
Laskari K
Lazar C
Minoia F
Nigrovic PA
Oliveira Ramos F
Ozen S
Quartier P
Ruscitti P
Sag E
Savic S
Truchetet ME
Vastert SJ
Wilhelmer TC
Wouters C
Carmona L
De Benedetti F
author Fautrel B
author_facet Fautrel B
Mitrovic S
De Matteis A
Bindoli S
Antón J
Belot A
Bracaglia C
Constantin T
Dagna L
Di Bartolo A
Feist E
Foell D
Gattorno M
Georgin-Lavialle S
Giacomelli R
Grom AA
Jamilloux Y
Laskari K
Lazar C
Minoia F
Nigrovic PA
Oliveira Ramos F
Ozen S
Quartier P
Ruscitti P
Sag E
Savic S
Truchetet ME
Vastert SJ
Wilhelmer TC
Wouters C
Carmona L
De Benedetti F
author_role author
author2 Mitrovic S
De Matteis A
Bindoli S
Antón J
Belot A
Bracaglia C
Constantin T
Dagna L
Di Bartolo A
Feist E
Foell D
Gattorno M
Georgin-Lavialle S
Giacomelli R
Grom AA
Jamilloux Y
Laskari K
Lazar C
Minoia F
Nigrovic PA
Oliveira Ramos F
Ozen S
Quartier P
Ruscitti P
Sag E
Savic S
Truchetet ME
Vastert SJ
Wilhelmer TC
Wouters C
Carmona L
De Benedetti F
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Still's disease
adult-onset
arthritis
juvenile
macrophage activation syndrome
biological therapy
inflammation
topic Still's disease
adult-onset
arthritis
juvenile
macrophage activation syndrome
biological therapy
inflammation
description Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. Methods In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly. Results The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still's disease. Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-gamma inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still's disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement. Conclusion These recommendations are the first consensus for the diagnosis and management of children and adults with Still's disease.
publishDate 2024
dc.date.none.fl_str_mv 2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=26900
url https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=26900
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv ELSEVIER
publisher.none.fl_str_mv ELSEVIER
dc.source.none.fl_str_mv ANNALS OF THE RHEUMATIC DISEASES
ISSN: 00034967
ISSNe: 14682060
reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname:Fundació Sant Joan de Déu
instname_str Fundació Sant Joan de Déu
reponame_str r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
collection r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,811543

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