Clinical manifestations and treatment of antisynthetase syndrome

Abstract

Antisynthetase syndrome (ASyS) is an autoimmune disease clinically manifested most often by interstitial lung disease, myositis, and arthritis. Raynaud's syndrome, fever, and rashes are also commonly seen. This syndrome is characterized by the highly specific presence of antibodies against various aminoacyl transfer RNA synthetases, including Jo-1 and others. In this chapter, we provide an overview of ASyS, including pathogenesis, common clinical manifestations, and treatment strategies. We discuss the spectrum of disease seen with specific antisynthetase antibodies and examine the differences in phenotype between patients with different antisynthetase antibodies. We outline common treatment strategies, which should generally target the most severe and life- or organ-threatening disease manifestations. Finally, we discuss short- and long-term prognosis in ASyS.

Keywords: Anti-Jo-1; Antisynthetase antibody; Antisynthetase syndrome; Arthritis; Idiopathic inflammatory myopathy; Interstitial lung disease; Mechanic's hands.