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.

Highlights

  • Antisynthetase syndrome (ASSD) is a rare connective tissue disease that affects the skin, joints, muscles, and lungs [1]

  • Anti-Jo1-positive patients had higher rates of arthritis when compared with other aminoacyl tRNA synthetases (ARS) (p < 0.01), with the exception of anti-OJ ARS, at onset (p = 0.06)

  • Even if arthritis was substantially more frequent in anti-Jo1 positive patients, the characteristics were similar independently to the underlying ARS specificity

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Summary

Introduction

Antisynthetase syndrome (ASSD) is a rare connective tissue disease that affects the skin, joints, muscles, and lungs [1]. The most important advances in ASSD have been mainly obtained in anti-Jo1 syndrome [2]. In this condition, the timing of the appearance of different manifestations is heterogeneous; isolated arthritis, generally similar to Rheumatoid Arthritis (RA), is the most frequent presentation [3,4,5,6], and anti-Ro antibodies co-occur in 50% of cases [7]. We have limited information regarding the clinical presentation pattern and evolution associated with other anti-aminoacyl tRNA synthetase antibodies (ARS). The aim of this study is to fully describe the effects of ARS in the clinical spectrum time course of ASSD and establish whether this condition is a unique syndrome or a group of different diseases

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