Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course.

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ID: 106533
2019
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Abstract
Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
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Authors Cavagna, Lorenzo;Trallero-Araguás, Ernesto;Meloni, Federica;Cavazzana, Ilaria;Rojas-Serrano, Jorge;Feist, Eugen;Zanframundo, Giovanni;Morandi, Valentina;Meyer, Alain;Pereira da Silva, Jose Antonio;Matos Costa, Carlo Jorge;Molberg, Oyvind;Andersson, Helena;Codullo, Veronica;Mosca, Marta;Barsotti, Simone;Neri, Rossella;Scirè, Carlo;Govoni, Marcello;Furini, Federica;Lopez-Longo, Francisco Javier;Martinez-Barrio, Julia;Schneider, Udo;Lorenz, Hanns-Martin;Doria, Andrea;Ghirardello, Anna;Ortego-Centeno, Norberto;Confalonieri, Marco;Tomietto, Paola;Pipitone, Nicolò;Rodriguez Cambron, Ana Belen;Blázquez Cañamero, María Ángeles;Voll, Reinhard Edmund;Wendel, Sarah;Scarpato, Salvatore;Maurier, Francois;Limonta, Massimiliano;Colombelli, Paolo;Giannini, Margherita;Geny, Bernard;Arrigoni, Eugenio;Bravi, Elena;Migliorini, Paola;Mathieu, Alessandro;Piga, Matteo;Drott, Ulrich;Delbrueck, Christiane;Bauhammer, Jutta;Cagnotto, Giovanni;Vancheri, Carlo;Sambataro, Gianluca;De Langhe, Ellen;Sainaghi, Pier Paolo;Monti, Cristina;Gigli Berzolari, Francesca;Romano, Mariaeva;Bonella, Francesco;Specker, Christof;Schwarting, Andreas;Villa Blanco, Ignacio;Selmi, Carlo;Ceribelli, Angela;Nuno, Laura;Mera-Varela, Antonio;Perez Gomez, Nair;Fusaro, Enrico;Parisi, Simone;Sinigaglia, Luigi;Del Papa, Nicoletta;Benucci, Maurizio;Cimmino, Marco Amedeo;Riccieri, Valeria;Conti, Fabrizio;Sebastiani, Gian Domenico;Iuliano, Annamaria;Emmi, Giacomo;Cammelli, Daniele;Sebastiani, Marco;Manfredi, Andreina;Bachiller-Corral, Javier;Sifuentes Giraldo, Walter Alberto;Paolazzi, Giuseppe;Saketkoo, Lesley Ann;Giorgi, Roberto;Salaffi, Fausto;Cifrian, Jose;Caporali, Roberto;Locatelli, Francesco;Marchioni, Enrico;Pesci, Alberto;Dei, Giulia;Pozzi, Maria Rosa;Claudia, Lomater;Distler, Jorg;Knitza, Johannes;Schett, George;Iannone, Florenzo;Fornaro, Marco;Franceschini, Franco;Quartuccio, Luca;Gerli, Roberto;Bartoloni, Elena;Bellando Randone, Silvia;Zampogna, Giuseppe;Gonzalez Perez, Montserrat I;Mejia, Mayra;Vicente, Esther;Triantafyllias, Konstantinos;Lopez-Mejias, Raquel;Matucci-Cerinic, Marco;Selva-O'Callaghan, Albert;Castañeda, Santos;Montecucco, Carlomaurizio;Gonzalez-Gay, Miguel Angel;
Journal journal of clinical medicine
Year 2019
DOI
E2013
URL
Keywords
interstitial lung disease arthritis myositis antisynthetase antibodies antisynthetase syndrome

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