BACKGROUND: With the advent and advancement of autoantibodies, there has been progress in the diagnosis, prognosis, and treatment of rheumatologic diseases. Antisynthetase syndrome (ASS) is a great example of a disease that initially was described as arthritis, myositis, interstitial lung disease, mechanic’s hands, Raynaud’s phenomenon, and fever in the presence of the anti-JO-1 antibody, but nowadays it presents with a different spectrum related to new antibodies.

CASE REPORT: We describe 4 patients with antisynthetase syndrome who were diagnosed with antibodies specific for myositis associated with different clinical findings. All patients responded to immunosuppressive therapy, and rituximab was the most used.

CONCLUSIONS: It is necessary to search for specific autoantibodies related to the syndrome in suspected clinical cases and in other rheumatological diseases refractory to the usual treatment.

Keywords: Amino Acyl-tRNA Synthetases, Autoantibodies, myositis, Glucocorticoids, Immunosuppressive Agents, Methotrexate, Prednisone, Rare Diseases