Antisynthetase syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Synonyms and keywords:

Overview

Antisynthetase syndrome, an uncommon autoimmune disorder, is characterized by antibodies against aminoacyl-transfer ribonucleic acid synthetases, with various combinations of three cardinal manifestations of interstitial lung disease (ILD), polymyositis, and arthritis. Other features of antisynthetase syndrome may include pyrexia of unknown origin, mechanic's hand and Raynaud's phenomenon.

Historical Perspective

Antisynthetase syndrome was first described by Marguerie et al as a triad of polymyositis, interstitial lung disease (ILD), and the presence of myositis-specific autoantibodies to aminoacyl-transfer ribonucleic acid synthetases.

Differentiating Antisynthetase syndrome from other Diseases

Antisynthetase syndrome may be misdiagnosed as idiopathic interstitial lung disease (ILD) or inflammatory myopathy. This is due to the rarity and lack of awareness of the disease, as well as due to the lack of

Most cases of AS are either misdiagnosed as idiopathic ILD or inflammatory myopathy not only due to the lack of awareness of the disease entity but also due to the lack of facilities for detection of anti-ARS antibodies.