Nguyen Nam, Leclair Valérie, Kaedbey Rayan, Hudson Marie
Faculty of Medicine and Health Sciences, McGill University, Montreal, QC, Canada.
Division of Rheumatology, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada.
SAGE Open Med Case Rep. 2025 Apr 29;13:2050313X251336022. doi: 10.1177/2050313X251336022. eCollection 2025.
Anti-synthetase syndrome is a subset of idiopathic inflammatory myopathies, for which refractory disease can be difficult to treat. Daratumumab, an anti-CD38 monoclonal antibody primarily used in hematologic malignancies, has recently shown promise in very few case reports of refractory idiopathic inflammatory myopathies. We present a case of a 44-year-old man with anti-Jo1 anti-synthetase syndrome with concurrent anti-Ro52, in which myositis remained refractory to multiple immunosuppressants. Despite treatment with daratumumab, the patient did not respond and ultimately succumbed to infection. This case underscores the need for further research to better define the role of daratumumab in idiopathic inflammatory myopathies and identify predictors of treatment response.
抗合成酶综合征是特发性炎性肌病的一个亚型,其难治性疾病可能难以治疗。达雷妥尤单抗是一种主要用于血液系统恶性肿瘤的抗CD38单克隆抗体,最近在极少数难治性特发性炎性肌病的病例报告中显示出前景。我们报告一例44岁男性,患有抗Jo1抗合成酶综合征并伴有抗Ro52,其肌炎对多种免疫抑制剂均难治。尽管使用了达雷妥尤单抗治疗,但患者无反应,最终死于感染。该病例强调需要进一步研究,以更好地明确达雷妥尤单抗在特发性炎性肌病中的作用,并确定治疗反应的预测指标。