Feed

Antisynthetase syndrome (ASS) is a systemic autoimmune disorder classified as a subtype of the idiopathic inflammatory myopathies (IIM). The condition is defined by the presence of mutually exclusive autoantibodies directed against an aminoacyl-tRNA synthetase along with typical clinical manifestations, including myositis, Raynaud's phenomenon, arthritis, skin lesions such as mechanic hands, or interstitial lung disease (ILD). Anti-synthetase syndrome associated interstitial lung disease (ASS-ILD) can range from mild forms to rapidly progressive disease, which may lead to chronic pulmonary damage if misdiagnosed or inadequately treated. Patients with IIM carry an increased risk of developing neoplasms, most commonly adenocarcinoma, but not lymphoma or other hematologic malignancies. However, data on the association between ASS and malignancy remain very limited. We report the case of a patient with anti-PL-12 antisynthetase syndrome who subsequently developed NK/T-cell non-Hodgkin lymphoma, supplemented by a review of the pertinent literature.