Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Oct;5(3):178-191.
doi: 10.1177/2397198320902667. Epub 2020 Feb 18.

Antisynthetase syndrome: A distinct disease spectrum

Kun Huang  1 , Rohit Aggarwal  2
Affiliations
Review

Antisynthetase syndrome: A distinct disease spectrum

Kun Huang et al. J Scleroderma Relat Disord. 2020 Oct.

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.

Keywords: Inflammatory myositis; antisynthetase syndrome; clinicoserological spectrum; interstitial lung disease; myositis-specific antibodies.

PubMed Disclaimer

Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
A proposed treatment algorithm for myositis-associated ILD (adapted from Oddis and Aggarwal).
See this image and copyright information in PMC

References

    1. Ghirardello A, Bassi N, Palma L, et al.. Autoantibodies in polymyositis and dermatomyositis. Curr Rheumatol Rep 2013; 15: 335. - PubMed
    1. Targoff IN. Idiopathic inflammatory myopathy: autoantibody update. Curr Rheumatol Rep 2002; 4(5): 434–441. - PubMed
    1. Koenig M, Fritzler MJ, Targoff IN, et al.. Heterogeneity of autoantibodies in 100 patients with autoimmune myositis: insights into clinical features and outcomes. Arthritis Res Ther 2007; 9(4): R78. - PMC - PubMed
    1. Nishikai M, Reichlin M. Heterogeneity of precipitating antibodies in polymyositis and dermatomyositis. Characterization of the Jo-1 antibody system. Arthritis Rheum 1980; 23: 881–888. - PubMed
    1. Mathews MB, Reichlin M, Hughes GR, et al.. Anti-threonyl-tRNA synthetase, a second myositis-related autoantibody. J Exp Med 1984; 160(2): 420–434. - PMC - PubMed