Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course
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 , Matos Costa Carlo , Molberg Oyvind , Andersson Helena , Codullo Veronica , Mosca Marta , Barsotti Simone , Neri Rossella , Scirè Carlo , Govoni Marcello , Furini Federica , Lopez-Longo Francisco , Martinez-Barrio Julia , Schneider Udo , Lorenz Hanns-Martin , Doria Andrea , Ghirardello Anna , Ortego-Centeno Norberto , Confalonieri Marco , Tomietto Paola , Pipitone Nicolò , Rodriguez Cambron Ana , Blázquez Cañamero María , Voll Reinhard , 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 , 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 , Riccieri Valeria , Conti Fabrizio , Sebastiani Gian , Iuliano Annamaria , Emmi Giacomo , Cammelli Daniele , Sebastiani Marco , Manfredi Andreina , Bachiller-Corral Javier , Sifuentes Giraldo Walter , Paolazzi Giuseppe , Saketkoo Lesley , Giorgi Roberto , Salaffi Fausto , Cifrian Jose , Caporali Roberto , Locatelli Francesco , Marchioni Enrico , Pesci Alberto , Dei Giulia , Pozzi Maria , 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 , Mejia Mayra , Vicente Esther , Triantafyllias Konstantinos , Lopez-Mejias Raquel , Matucci-Cerinic Marco , Selva-O’Callaghan Albert , Castañeda Santos , Montecucco Carlomaurizio , Gonzalez-Gay Miguel 
Published: November 2019

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.