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Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

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dc.contributor.authorCavagna, Lorenzo
dc.contributor.authorMeloni, Federica
dc.contributor.authorMeyer, Alain
dc.contributor.authorSambataro, Gianluca
dc.contributor.authorBelliato, Mirko
dc.contributor.authorDe Langhe, Ellen
dc.contributor.authorCavazzana, Ilaria
dc.contributor.authorPipitone, Nicolò
dc.contributor.authorTriantafyllias, Konstantinos
dc.contributor.authorMosca, Marta
dc.contributor.authorBarsotti, Simone
dc.contributor.authorZampogna, Giuseppe
dc.contributor.authorBiglia, Alessandro
dc.contributor.authorEmmi, Giacomo
dc.contributor.authorDe Visser, Marianne
dc.contributor.authorVan Der Kooi, Anneke
dc.contributor.authorParronchi, Paola
dc.contributor.authorHirschi, Sandrine
dc.contributor.authorda Silva, Jose Antonio Pereira
dc.contributor.authorScirè, Carlo Alberto
dc.contributor.authorFurini, Federica
dc.contributor.authorGiannini, Margherita
dc.contributor.authorMartinez Gonzalez, Olga
dc.contributor.authorDamian, Laura
dc.contributor.authorPiette, Yves
dc.contributor.authorSmith, Vanessa
dc.contributor.authorMera-Valera, Antonio
dc.contributor.authorBachiller-Corral, Javier
dc.contributor.authorCabezas Rodriguez, Ivan
dc.contributor.authorBrandy-Garcia, Anahy M.
dc.contributor.authorMaurier, François
dc.contributor.authorPerrin, Julie
dc.contributor.authorGonzalez-Moreno, Juan
dc.contributor.authorDrott, Ulrich
dc.contributor.authorDelbruck, Christiane
dc.contributor.authorSchwarting, Andreas
dc.contributor.authorArrigoni, Eugenio
dc.contributor.authorSebastiani, Gian Domenico
dc.contributor.authorIuliano, Annamaria
dc.contributor.authorNannini, Carlotta
dc.contributor.authorQuartuccio, Luca
dc.contributor.authorRodriguez Cambron, Ana B.
dc.contributor.authorBlázquez Cañamero, Maria Á.
dc.contributor.authorVilla Blanco, Ignacio
dc.contributor.authorCagnotto, Giovanni
dc.contributor.authorPesci, Alberto
dc.contributor.authorLuppi, Francesco
dc.contributor.authorDei, Giulia
dc.contributor.authorRomero Bueno, Fredeswinda Isabel
dc.contributor.authorFranceschini, Franco
dc.contributor.authorChiapparoli, Ilaria
dc.contributor.authorZanframundo, Giovanni
dc.contributor.authorLettieri, Sara
dc.contributor.authorDe Stefano, Ludovico
dc.contributor.authorCutolo, Maurizio
dc.contributor.authorMathieu, Alessandro
dc.contributor.authorPiga, Matteo
dc.contributor.authorPrieto-González, Sergio
dc.contributor.authorMoraes-Fontes, Maria Francisca
dc.contributor.authorFonseca, João Eurico
dc.contributor.authorJovani, Vega
dc.contributor.authorRiccieri, Valeria
dc.contributor.authorSantaniello, Alessandro
dc.contributor.authorMontfort, Stephen
dc.contributor.authorBilocca, David
dc.contributor.authorErre, Gian Luca
dc.contributor.authorBartoloni, Elena
dc.contributor.authorGerli, Roberto
dc.contributor.authorMonti, M. Cristina
dc.contributor.authorLorenz, Hanns M.
dc.contributor.authorSambataro, Domenico
dc.contributor.authorBellando Randone, Silvia
dc.contributor.authorSchneider, Udo
dc.contributor.authorValenzuela, Claudia
dc.contributor.authorLopez-Mejias, Raquel
dc.contributor.authorCifrian, Jose
dc.contributor.authorMejia, Mayra
dc.contributor.authorGonzalez Perez, Monserrat-Ixchel
dc.contributor.authorWendel, Sarah
dc.contributor.authorFornaro, Marco
dc.contributor.authorDe Luca, Giacomo
dc.contributor.authorOrsolini, Giovanni
dc.contributor.authorRossini, Maurizio
dc.contributor.authorDieude, Philippe
dc.contributor.authorKnitza, Johannes
dc.contributor.authorCastañeda, Santos
dc.contributor.authorVoll, Reinhard E.
dc.contributor.authorRojas-Serrano, Jorge
dc.contributor.authorValentini, Adele
dc.contributor.authorVancheri, Carlo
dc.contributor.authorMatucci-Cerinic, Marco
dc.contributor.authorFeist, Eugen
dc.contributor.authorCodullo, Veronica
dc.contributor.authorIannone, Florenzo
dc.contributor.authorDistler, Jorg H.
dc.contributor.authorMontecucco, Carlomaurizio
dc.contributor.authorGonzalez-Gay, Miguel A.
dc.date.accessioned2023-06-09T14:05:40Z
dc.date.available2023-06-09T14:05:40Z
dc.date.issued2022
dc.description© Copyright Clinical and Experimental Rheumatology 2022.pt_PT
dc.description.abstractObjectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationClin Exp Rheumatol. 2022 Feb;40(2):274-283pt_PT
dc.identifier.doi10.55563/clinexprheumatol/di1083pt_PT
dc.identifier.eissn1593-098X
dc.identifier.issn0392-856X
dc.identifier.urihttp://hdl.handle.net/10451/58144
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherPacini Editore SpApt_PT
dc.relation.publisherversionhttps://www.clinexprheumatol.org/default.asppt_PT
dc.subjectMelanoma differentiation-associated protein 5 antibodypt_PT
dc.subjectRapidly progressive interstitial lung diseasespt_PT
dc.subjectIdiopathic inflammatory myopathiespt_PT
dc.titleClinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodiespt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage283pt_PT
oaire.citation.issue2pt_PT
oaire.citation.startPage274pt_PT
oaire.citation.titleClinical and Experimental Rheumatologypt_PT
oaire.citation.volume40pt_PT
person.familyNameMoraes-Fontes
person.familyNameFonseca
person.givenNameMaria Francisca
person.givenNameJoão
person.identifier.ciencia-id791A-696C-7248
person.identifier.ciencia-idF310-B85D-57C7
person.identifier.orcid0000-0002-8917-6592
person.identifier.orcid0000-0003-1432-3671
person.identifier.scopus-author-id7101983519
rcaap.rightsrestrictedAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication1c0556ff-4931-4bc7-bdc4-e643f7a0f97c
relation.isAuthorOfPublication1772dc12-7c55-4c76-ae2d-c23270172480
relation.isAuthorOfPublication.latestForDiscovery1c0556ff-4931-4bc7-bdc4-e643f7a0f97c

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