Ye Wen-Zheng, Peng Shan-Shan, Hu You-Hong, Fang Mu-Ping, Xiao Yu
Department of Dermatology, The Central Hospital of Xiaogan, Xiaogan Hospital Affiliated to Wuhan University of Science and Technology, Xiaogan 432000, Hubei Province, China.
Department of Respiratory and Critical Care Medicine, The Central Hospital of Xiaogan, Xiaogan Hospital Affiliated to Wuhan University of Science and Technology, Xiaogan 432000, Hubei Province, China.
World J Clin Cases. 2023 Aug 16;11(23):5580-5588. doi: 10.12998/wjcc.v11.i23.5580.
Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis (DM) manifestations without muscle weakness. Anti-melanoma differentiation-associated gene 5 (anti-MDA5) and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease, and they exhibit an immensely high half-year mortality.
We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM. Although we aggressively treated these patients similarly, but they all exhibited different prognoses. We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.
Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively, thus, the patient's prognosis can be significantly modified.
临床无肌病性皮肌炎表现为各种皮肌炎(DM)表现但无肌无力。抗黑色素瘤分化相关基因5(抗MDA5)和抗Ro52抗体双阳性的临床无肌病性DM患者发生快速进展性间质性肺病的风险很高,且半年死亡率极高。
我们报告了3例抗MDA5和抗Ro52抗体双阳性的DM患者,并回顾了以往关于抗MDA5和抗Ro52抗体双阳性DM之间联系的研究。尽管我们对这些患者进行了类似的积极治疗,但他们都表现出不同的预后。我们回顾了临床皮肤皮疹的重要性以及抗MDA5和抗Ro52双阳性相关DM的发病机制和治疗。
抗MDA5抗Ro52抗体双阳性的DM患者应早期准确诊断并积极治疗,从而可显著改善患者预后。