Ling Gui-Chen, Su Chang, Guo Ying-Ao, Qiu Xia, Liu Jia-Wei, Xiao Min, Xiao Yu-Ya, Yang Shuo, Zhang Jian-Yong, Xie Jing-Jing
The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, Shenzhen, Guangdong, China.
The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, Shenzhen, Guangdong, China.
Front Pharmacol. 2024 Oct 30;15:1472667. doi: 10.3389/fphar.2024.1472667. eCollection 2024.
Dermatomyositis (DM) positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, mainly when linked with rapidly progressive interstitial lung disease (RP-ILD), is considered a refractory disease. Our report describes a critical case of clinically amyopathic dermatomyositis (CADM) with RP-ILD that tested positive for both anti-MDA5 and anti-Ro-52 antibodies. The patient showed a limited response to a combined therapy regimen of prednisone, iguratimod, and tacrolimus. However, after adjunct therapy with mycophenolate mofetil (MMF), the patient's condition was controlled, his serum KL-6 levels decreased, and anti-MDA5 antibodies became negative. During the 68-week follow-up, the patient's condition remained stable, with a satisfactory quality of life. This report also discusses the potential role of inflammatory cytokines in the pathophysiology of CADM and RP-ILD. Further research is required to confirm these results and investigate the application of MMF in maintenance therapy for CADM-associated RP-ILD.
抗黑色素瘤分化相关基因5（MDA5）抗体阳性的皮肌炎（DM），主要是与快速进展性间质性肺病（RP-ILD）相关联时，被认为是一种难治性疾病。我们的报告描述了一例临床无肌病性皮肌炎（CADM）合并RP-ILD的重症病例，该病例抗MDA5和抗Ro-52抗体均呈阳性。患者对泼尼松、艾拉莫德和他克莫司的联合治疗方案反应有限。然而，在加用霉酚酸酯（MMF）辅助治疗后，患者病情得到控制，血清KL-6水平下降，抗MDA5抗体转阴。在68周的随访期间，患者病情保持稳定，生活质量令人满意。本报告还讨论了炎性细胞因子在CADM和RP-ILD病理生理学中的潜在作用。需要进一步研究以证实这些结果，并探讨MMF在CADM相关RP-ILD维持治疗中的应用。
