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. 2019 Nov 18;8(11):2013.
doi: 10.3390/jcm8112013.

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

Lorenzo Cavagna  1 , Ernesto Trallero-Araguás  2 , Federica Meloni  3 , Ilaria Cavazzana  4 , Jorge Rojas-Serrano  5 , Eugen Feist  6 , Giovanni Zanframundo  1 , Valentina Morandi  1 , Alain Meyer  7   8 , Jose Antonio Pereira da Silva  9 , Carlo Jorge Matos Costa  9 , Oyvind Molberg  10 , Helena Andersson  10 , Veronica Codullo  11 , Marta Mosca  12 , Simone Barsotti  12 , Rossella Neri  12 , Carlo Scirè  13 , Marcello Govoni  13 , Federica Furini  13 , Francisco Javier Lopez-Longo  14 , Julia Martinez-Barrio  14 , Udo Schneider  6 , Hanns-Martin Lorenz  15 , Andrea Doria  16 , Anna Ghirardello  16 , Norberto Ortego-Centeno  17 , Marco Confalonieri  18 , Paola Tomietto  19 , Nicolò Pipitone  20 , Ana Belen Rodriguez Cambron  21 , María Ángeles Blázquez Cañamero  21 , Reinhard Edmund Voll  22 , Sarah Wendel  22 , Salvatore Scarpato  23 , Francois Maurier  24 , Massimiliano Limonta  25 , Paolo Colombelli  26 , Margherita Giannini  8 , Bernard Geny  8 , Eugenio Arrigoni  27 , Elena Bravi  27 , Paola Migliorini  28 , Alessandro Mathieu  29 , Matteo Piga  29 , Ulrich Drott  30 , Christiane Delbrueck  30 , Jutta Bauhammer  31 , Giovanni Cagnotto  32 , Carlo Vancheri  33 , Gianluca Sambataro  33 , Ellen De Langhe  34 , Pier Paolo Sainaghi  35 , Cristina Monti  36 , Francesca Gigli Berzolari  36 , Mariaeva Romano  37 , Francesco Bonella  38 , Christof Specker  39 , Andreas Schwarting  40 , Ignacio Villa Blanco  41 , Carlo Selmi  42 , Angela Ceribelli  42 , Laura Nuno  43 , Antonio Mera-Varela  44 , Nair Perez Gomez  44 , Enrico Fusaro  45 , Simone Parisi  45 , Luigi Sinigaglia  46 , Nicoletta Del Papa  46 , Maurizio Benucci  47 , Marco Amedeo Cimmino  48 , Valeria Riccieri  49 , Fabrizio Conti  49 , Gian Domenico Sebastiani  50 , Annamaria Iuliano  50 , Giacomo Emmi  51 , Daniele Cammelli  52 , Marco Sebastiani  53 , Andreina Manfredi  53 , Javier Bachiller-Corral  54 , Walter Alberto Sifuentes Giraldo  54 , Giuseppe Paolazzi  55 , Lesley Ann Saketkoo  56 , Roberto Giorgi  57 , Fausto Salaffi  58 , Jose Cifrian  59 , Roberto Caporali  60 , Francesco Locatelli  1 , Enrico Marchioni  61 , Alberto Pesci  62 , Giulia Dei  62 , Maria Rosa Pozzi  62 , Lomater Claudia  63 , Jorg Distler  64 , Johannes Knitza  64 , George Schett  64 , Florenzo Iannone  65 , Marco Fornaro  65 , Franco Franceschini  4 , Luca Quartuccio  66 , Roberto Gerli  67 , Elena Bartoloni  67 , Silvia Bellando Randone  68 , Giuseppe Zampogna  69 , Montserrat I Gonzalez Perez  5 , Mayra Mejia  5 , Esther Vicente  70 , Konstantinos Triantafyllias  71 , Raquel Lopez-Mejias  72 , Marco Matucci-Cerinic  68 , Albert Selva-O'Callaghan  2 , Santos Castañeda  70   73 , Carlomaurizio Montecucco  1 , Miguel Angel Gonzalez-Gay  72
Affiliations

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

Lorenzo Cavagna et al. J Clin Med. .

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.

Keywords: antisynthetase antibodies; antisynthetase syndrome; arthritis; interstitial lung disease; myositis.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
Triad findings cluster (in percentage) at disease onset and last follow-up. Significant differences between groups were evaluated considering anti-Jo1 positive patients as the reference group. Chi-square or Fisher exact test were used as appropriate (significance threshold: p < 0.0125, reported as *** p < 0.001, ** p < 0.01). Legend: ILD, interstitial lung disease.
Figure 2
Figure 2
The Kaplan–Meier survival curve in our cohort of antisynthetase syndrome patients (established range: 0–60 months) with Log-rank test at 12, 24 and 60 months. Mo: months.
See this image and copyright information in PMC

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