Abstract
Abstract We report a male patient who had refractory idiopathic inflammatory myopathy (IIM) presented with antisynthetase syndrome, being treated by potent immunosuppressants for years, developed Epstein-Barr virus (EBV)-associated lymphoma. Despite the stepping down of the immunosuppressives and active lymphoma therapy, the patient died. On top of the typical association of IIM and malignancy, rare EBV-associated tumors related to EBV infection secondary to the use of potent immunosuppressive therapies could occur. Further investigations are advisable if there are new symptoms and signs or in refractory IIM cases. This report serves as a diagnostic alert that the causation by EBV infection in unusual tumors found in patients with IIM should be considered, as both the treatment and prognosis may differ. A balance between the risks and benefits of immunosuppressive therapies should always be achieved.
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