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Antisynthetase syndrome: A distinct disease spectrum

Journal

JOURNAL OF SCLERODERMA AND RELATED DISORDERS
Volume 5, Issue 3, Pages 178-191

Publisher

SAGE PUBLICATIONS LTD
DOI: 10.1177/2397198320902667

Keywords

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

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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.

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