Antisynthetase myopathy

Antisynthetase syndrome: not just an inflammatory myopathy.

In recent years, antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibodies to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, "mechanic's hands," Rayna...

Myofiber HLA-DR expression is a distinctive biomarker for antisynthetase-associated myopathy

OBJECTIVES To assess the value of major histocompatibility complex (MHC) class II antigen (HLA-DR) expression to distinguish anti-synthetase myopathy (ASM) from dermatomyositis (DM). METHODS Muscle biopsies from patients with ASM (n = 33), DM without anti-synthetase antibodies (ASAb) (n = 17), and normal muscle biopsy (n = 10) were first reviewed. ASAb included anti-Jo1 (26/33), anti-PL12 (4/...

Antisynthetase syndrome

The Antisynthetase Syndrome

The antisynthetase syndrome (ASS) was first described by Marguerie and coworkers in 1990 as a triad of polymyositis, diffuse interstitial lung disease (ILD) and serum autoantibodies to aminoacyl transfer RNA synthetases (anti-ARS) (1). Later, cohort studies have indicated that 20-25 % of patients diagnosed with polymyositis (PM) or dermatomyositis (DM) have antiARS antibodies (2-4). In most cas...

Drug-induced antisynthetase syndrome.

We present a case of antisynthetase syndrome whose predominant feature was fibrosing alveolitis and which may have been drug-induced. This responded well to steroids and cyclophosphamide.