Antisynthetase Syndrome

Antisynthetase syndrome (ASS) is classified as a subset of idiopathic inflammatory myopathies (IIM). Its clinical manifestations encompass more than muscular involvement, including interstitial lung disease (ILD), arthritis, fever, characteristic cutaneous lesions, and Raynaud’s phenomenon. Additionally, a distinctive muscle biopsy pattern is often observed. The diagnosis is based on the detection of myositis-specific antibodies targeting aminoacyl tRNA synthetase, supported by relevant clinical, pathological, and radiological findings. To date, eight antisynthetase antibodies (ASA) have been identified, with antibodies against histidyl-tRNA synthetase (anti-Jo-1) being the most prevalent. While explicit diagnostic or classification criteria for ASS remain unestablished, the presence of a primary clinical feature coupled with the corresponding autoantibody is generally indicative of the syndrome.