Anti‐Jo1 autoantibodies, from clinic to the bench

Abstract Introduction Idiopathic inflammatory myopathies (IIM) also known as myositis, is a chronic autoimmune disease which affects muscles, skin, joints and lung. One of the most common autoantibodies with a prevalence of 25‐35%, is the anti‐Jo‐1 autoantibodies, targeting the histidyl‐transfer RNA synthetase (HisRS). These autoantibodies are strongly associated with a distinct clinical phenotype called anti‐synthetase syndrome (ASyS) that includes interstitial lung disease (ILD), myositis, arthritis and Raynaud's phenomenon. The mechanism of how autoantibodies are produced and how they contribute to disease pathogenesis is unclear. Areas Covered In this review we will discuss the clinical manifestations associated with Jo‐1 autoantibodies, and the association with clinical features. Moreover, we will also review the molecular characteristics of the antibodies and how they affect the cells of the immune system in order to understand possible disease mechanisms.