Abstract：
Anti-melanoma differentiation-associated gene 5-positive (Anti-MDA5) dermatomyositis (DM) is an aggressive phenotype of DM associated with rapidly progressive interstitial lung disease (RP-ILD). It is a rare condition that carries high mortality. Diagnosis and management of patients with anti-MDA5 DM RP-ILD presents several challenges, including uncertainty around treatment algorithms and a lack of evidence to inform practice. This case report of a patient with anti-MDA5 DM RP-ILD highlights these challenges, emphasising the fulminant course of this disease despite aggressive immunosuppression. Further research is required to guide management and to minimise morbidity and mortality, and greater awareness of the condition is required to minimise delays in diagnosis.
摘要：
抗黑素瘤分化相关基因5阳性（抗MDA5）皮肌炎（DM）是与快速进行性间质性肺病（RP-ILD）相关的DM的侵袭性表型。这是一种罕见的高死亡率疾病。抗MDA5DMRP-ILD患者的诊断和治疗面临着一些挑战，包括治疗算法的不确定性和缺乏指导实践的证据。一例抗MDA5DMRP-ILD患者的病例报告强调了这些挑战，强调这种疾病的暴发性过程，尽管积极的免疫抑制。需要进一步的研究来指导管理并将发病率和死亡率降至最低。并且需要提高对病情的认识，以最大程度地减少诊断的延迟。
