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Antisynthetase syndrome: A distinct disease spectrum

Kun Huang, Rohit Aggarwal

2020Journal of Scleroderma and Related Disorders77 citationsDOIOpen Access PDF

Abstract

The discovery of novel autoantibodies related to idiopathic inflammatory myopathies (collectively referred to as myositis) has not only advanced our understanding of the clinical, serological, and pathological correlation in the disease spectrum but also played a role in guiding management and prognosis. One group of the myositis-specific autoantibodies is anti-aminoacyl-tRNA synthetase (anti-ARS or anti-synthetase) which defines a syndrome with predominant interstitial lung disease, arthritis, and myositis. Autoantibodies to eight aminoacyl-tRNA synthetases have been identified with anti-Jo1 the most common in all of idiopathic inflammatory myopathies. Disease presentation and prognosis vary depending on which anti-aminoacyl-tRNA synthetase antibody is present. In this review, we will discuss the clinical characteristics, overlap features with other autoimmune diseases, prognostic factors, and management of the antisynthetase syndrome.

Topics & Concepts

Antisynthetase syndromeAutoantibodyMyositisMedicineDiseaseAminoacyl tRNA synthetaseInterstitial lung diseaseImmunologyAntibodyPathologyTransfer RNAInternal medicineLungBiologyGeneticsRNAGeneInflammatory Myopathies and DermatomyositisEosinophilic Disorders and SyndromesNeurogenetic and Muscular Disorders Research