Koyoda Sai K, Ezzy Fatema, Wahezi Dawn, Kumthekar Anand, Xie Xianhong, Tagoe Clement E, Ayesha Bibi
Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA.
Jacobi Medical Center, Department of Internal Medicine, Bronx, NY, USA.
BMC Rheumatol. 2025 Jan 9;9(1):5. doi: 10.1186/s41927-025-00455-5.
The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center.
A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer's exact test and a t test.
Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud's phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40-60%.
This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations.
抗黑色素瘤分化相关基因5（anti-MDA5）抗体阳性皮肌炎以其与快速进展性间质性肺病（RP-ILD）和溃疡性皮肤病变相关而闻名，常伴有或不伴有肌肉受累。本研究的目的是确定不同的临床和实验室特征，以用于评估美国一家学术中心不同种族队列的抗MDA5皮肌炎患者的疾病进展。
对2014年1月至2023年6月在我们机构住院的皮肌炎患者进行回顾性病历审查。通过费舍尔精确检验和t检验对数据进行分析。
在审查的195例皮肌炎患者中，22例MDA5抗体检测呈阳性，包括13例成人患者和9例儿科患者。肌炎在儿科患者中比在成人患者中更常见（p = 0.002）。RP-ILD在非洲裔成人患者（包括西班牙裔黑人及非西班牙裔黑人个体）中更常观察到（p = 0.04）。雷诺现象与ILD之间存在显著关联（p = 0.02）。13.6%的总死亡率比先前报道的40%-60%更有利。
本研究通过强调潜在的种族和人口统计学差异，以及描绘成人和儿科人群之间的异同，增强了我们对该疾病的理解。
