Cavagna, LorenzoMeloni, FedericaMeyer, AlainSambataro, GianlucaBelliato, MirkoDe Langhe, EllenCavazzana, IlariaPipitone, NicolòTriantafyllias, KonstantinosMosca, MartaBarsotti, SimoneZampogna, GiuseppeBiglia, AlessandroEmmi, GiacomoDe Visser, MarianneVan Der Kooi, AnnekeParronchi, PaolaHirschi, Sandrineda Silva, Jose Antonio PereiraScirè, Carlo AlbertoFurini, FedericaGiannini, MargheritaMartinez Gonzalez, OlgaDamian, LauraPiette, YvesSmith, VanessaMera-Valera, AntonioBachiller-Corral, JavierCabezas Rodriguez, IvanBrandy-Garcia, Anahy M.Maurier, FrançoisPerrin, JulieGonzalez-Moreno, JuanDrott, UlrichDelbruck, ChristianeSchwarting, AndreasArrigoni, EugenioSebastiani, Gian DomenicoIuliano, AnnamariaNannini, CarlottaQuartuccio, LucaRodriguez Cambron, Ana B.Blázquez Cañamero, Maria Á.Villa Blanco, IgnacioCagnotto, GiovanniPesci, AlbertoLuppi, FrancescoDei, GiuliaRomero Bueno, Fredeswinda IsabelFranceschini, FrancoChiapparoli, IlariaZanframundo, GiovanniLettieri, SaraDe Stefano, LudovicoCutolo, MaurizioMathieu, AlessandroPiga, MatteoPrieto-González, SergioMoraes-Fontes, Maria FranciscaFonseca, João EuricoJovani, VegaRiccieri, ValeriaSantaniello, AlessandroMontfort, StephenBilocca, DavidErre, Gian LucaBartoloni, ElenaGerli, RobertoMonti, M. CristinaLorenz, Hanns M.Sambataro, DomenicoBellando Randone, SilviaSchneider, UdoValenzuela, ClaudiaLopez-Mejias, RaquelCifrian, JoseMejia, MayraGonzalez Perez, Monserrat-IxchelWendel, SarahFornaro, MarcoDe Luca, GiacomoOrsolini, GiovanniRossini, MaurizioDieude, PhilippeKnitza, JohannesCastañeda, SantosVoll, Reinhard E.Rojas-Serrano, JorgeValentini, AdeleVancheri, CarloMatucci-Cerinic, MarcoFeist, EugenCodullo, VeronicaIannone, FlorenzoDistler, Jorg H.Montecucco, CarlomaurizioGonzalez-Gay, Miguel A.2023-06-092023-06-092022Clin Exp Rheumatol. 2022 Feb;40(2):274-2830392-856Xhttp://hdl.handle.net/10451/58144© Copyright Clinical and Experimental Rheumatology 2022.Objectives: 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.engMelanoma differentiation-associated protein 5 antibodyRapidly progressive interstitial lung diseasesIdiopathic inflammatory myopathiesjournal article10.55563/clinexprheumatol/di10831593-098X