Open Access

Lorenzo Cavagna, Ernesto Trallero-Araguás, Federica Meloni, Ilaria Cavazzana, Jorge Rojas-Serrano, Eugen Feist, Giovanni Zanframundo, Valentina Morandi, Alain Meyer, José António Pereira da Silva, Carlo Jorge Matos Costa, Oyvind Molberg, Helena Andersson, Veronica Codullo, Marta Mosca, Simone Barsotti, Rossella Neri, Carlo Alberto Scirè, Marcello Govoni, Federica Furini, Francisco Javier López-Longo, Julia Martínez-Barrio, Udo Schneider, Hanns-Martin Lorenz, Andrea Doria, Anna Ghirardello, Norberto Ortego-Centeno, Marco Confalonieri, Paola Tomietto, Nicolò Pipitone, Ana Belen Rodriguez Cambron, María Ángeles Blázquez Cañamero, Reinhard Voll, Sarah Wendel, Salvatore Scarpato, Francois Maurier, Massimiliano Limonta, Paolo Colombelli, Margherita Giannini, Bernard Geny, Eugenio Arrigoni, Elena Bravi, Paola Migliorini, Alessandro Mathieu, Matteo Piga, Ulrich Drott, Christiane Delbrück, Jutta Bauhammer, Giovanni Cagnotto, Carlo Vancheri, Gianluca Sambataro, Ellen De Langhe, Pier Paolo Sainaghi, Cristina Monti, Francesca Gigli Berzolari, Mariaeva Romano, Francesco Bonella, Christof Specker, Andreas Schwarting, Ignacio Villa Blanco, Carlo Selmi, Angela Ceribelli, Laura Nuno, Antonio Mera-Varela, Nair Perez Gomez, Enrico Fusaro, Simone Parisi, Luigi Sinigaglia, Nicoletta Del Papa, Maurizio Benucci, Marco Amedeo Cimmino, Valeria Riccieri, Fabrizio Conti, Gian Domenico Sebastiani, Annamaria Iuliano, Giacomo Emmi, Daniele Cammelli, Marco Sebastiani, Andreina Manfredi, Javier Bachiller Corral, Walter Alberto Sifuentes Giraldo, Giuseppe Paolazzi, Lesley Ann Saketkoo, Roberto Giorgi, Fausto Salaffi, Jose Cifrian, Roberto Caporali, Francesco Locatelli, Enrico Marchioni, Alberto Pesci, Giulia Dei, Maria Rosa Pozzi, Lomater Claudia, Jörg Hans Wilhelm Distler, Johannes Knitza, George Schett, Florenzo Iannone, Marco Fornaro, Franco Franceschini, Luca Quartuccio, Roberto Gerli, Elena Bartoloni, Silvia Bellando-Randone, Giuseppe Zampogna, Montserrat I. Gonzalez Perez, Mayra Mejia, Esther Vicente, Konstantinos Triantafyllias, Raquel López-Mejias, Marco Matucci-Cerinic, Albert Selva-O’Callaghan, Santos Castañeda, Carlomaurizio Montecucco, Miguel Ángel González-Gay

• 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.