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Serum EBV DNA was detected, and EBV\u2010encoded small RNA was positive in the tissue sample of LPD, indicating that EBV reactivation contributed to the pathogenesis of LPD in our case. The patient underwent chemotherapy, including rituximab, promptly after discontinuation of tacrolimus and cyclophosphamide, resulting in complete remission of the malignant lymphoma, and MDA5\u2010DM has not recurred with 3.5\u00a0mg\/d of prednisolone monotherapy. We reviewed 19 cases of OIIA\u2010LPD in patients with idiopathic inflammatory myopathies and herein report the first case of MDA5\u2010DM complicated with OIIA\u2010LPD. Among the 19 patients, 7 showed regression of LPD only following withdrawal of immunosuppressants, 9 took chemotherapy for LPD, and 5 died. It should be noted that patients with MDA5\u2010DM\u2010associated rapidly progressive interstitial lung disease could develop OIIA\u2010LPD because they receive aggressive immunosuppressive therapy.<\/jats:p>","DOI":"10.1111\/1756-185x.14608","type":"journal-article","created":{"date-parts":[[2023,2,15]],"date-time":"2023-02-15T10:20:46Z","timestamp":1676456446000},"page":"1172-1177","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Other iatrogenic immunodeficiency\u2010associated lymphoproliferative disorders in a patient with anti\u2010melanoma differentiation\u2010associated gene 5\u2010positive dermatomyositis: A case report and systematic literature review"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2530-6930","authenticated-orcid":false,"given":"Nozomi","family":"Nishimura","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology 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