Antisynthetase syndrome – a case report and literature review

Abstract

Antisynthetase (AS) syndrome is a rare idiopathic inflammatory myopathy characterized by myositis, Raynaud’s disease, fever, interstitial lung disease (ILD), ‘mechanic’s hands’ and arthropathy, and it is associated with the presence of antibodies against aminoacyl-tRNA synthetase (ARS), especially anti-Jo-1. We report the case of a 31-year-old Egyptian male who presented with fever, mechanic’s hands, subclinical myositis, arthritis and ILD. He had been treated previously at another hospital with broad-spectrum antibiotics but without response. He was extensively worked up for infections and found to have mycoplasma infection and strongly positive anti-Jo-1 and anti-Ro/SSA antibodies. AS syndrome was confirmed based on clinical features and laboratory results. The patient was treated with high-dose steroids and subsequently with mycophenolate mofetil and dramatically improved. This case highlights the importance of awareness of this rare condition, and that careful examination of the hands can be crucial as mechanics’s hands is a unique manifestation of AS syndrome, occurring in ≈20% of cases. Mycoplasma infection may have been a triggering factor in this case.