Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort.

IF 2.1 Q3 RHEUMATOLOGY
Sai K Koyoda, Fatema Ezzy, Dawn Wahezi, Anand Kumthekar, Xianhong Xie, Clement E Tagoe, Bibi Ayesha
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Abstract

Background: 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.

Methods: 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.

Results: 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%.

Conclusion: 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(抗mda5)抗体阳性的皮肌炎:不同种族患者队列的临床特征和结果
背景:抗黑色素瘤分化相关基因5(抗mda5)抗体阳性的皮肌炎因其与快速进展的间质性肺疾病(RP-ILD)和溃疡性皮肤病变相关而闻名,通常伴有或不伴有肌肉受累。本研究的目的是在美国一个学术中心确定不同种族的抗mda5皮肌炎患者的不同临床和实验室特征,以评估疾病进展。方法:对2014年1月至2023年6月在我院住院的皮肌炎患者进行回顾性图表分析。数据通过Fischer精确检验和t检验进行分析。结果:在195例皮肌炎患者中,22例MDA5抗体检测阳性,包括13例成人和9例儿科患者。肌炎在儿童患者中比在成人患者中更常见(p = 0.002)。RP-ILD更常见于非洲血统的成年患者(包括西班牙裔黑人和非西班牙裔黑人)(p = 0.04)。雷诺氏现象与ILD之间存在显著相关性(p = 0.02)。13.6%的总死亡率比先前报道的40-60%的死亡率更有利。结论:本研究通过强调潜在的种族和人口差异,以及描述成人和儿科人群之间的异同,增强了我们对该疾病的理解。
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来源期刊
BMC Rheumatology
BMC Rheumatology Medicine-Rheumatology
CiteScore
3.80
自引率
0.00%
发文量
73
审稿时长
15 weeks
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