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Clinical features and prognosis of idiopathic inflammatory myopathies with coexistent multiple myositis-specific antibodies
X. Liang1, J. Wu2, H. Ren3, M. Li4, C. Huang5, J.G. Guo6, D. Li7, J. Li8, J. Zhu9
- Department of Rheumatology and Immunology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
- Department of Rheumatology and Immunology, Ganzhou Hospital-Nanfang Hospital, Southern Medical University, Ganzhou, China.
- Southern Medical University, Guangzhou, China.
- Department of Rheumatology and Immunology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
- Department of Rheumatology and Immunology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
- Department of Rheumatology and Immunology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
- Department of Rheumatology and Immunology, Ganzhou Hospital-Nanfang Hospital, Southern Medical University, Ganzhou, China. lidongsheng2456@sina.com
- Department of Rheumatology and Immunology, Nanfang Hospital, Southern Medical University, Guangzhou, and Department of Traditional Chinese Internal Medicine, School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, China. lijuan@smu.edu.cn
- Department of Rheumatology and Immunology, Nanfang Hospital, and Department of Rheumatology and Immunology, Ganzhou Hospital-Nanfang HospitalSouthern Medical University, Guangzhou, China. jqzhujq@yeah.net
CER17426
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PMID: 38819961 [PubMed]
Received: 29/12/2023
Accepted : 11/03/2024
In Press: 30/05/2024
Abstract
OBJECTIVES:
This study aimed to evaluate the clinical significance of the coexistence of 2 or more myositis-specific antibodies (multiple MSAs) in adult patients with idiopathic inflammatory myopathies (IIM).
METHODS:
We assessed a cohort of 202 consecutive patients with IIM. Clinical features and survival rates were compared between patients with and without multiple MSAs.
RESULTS:
Of those 202 patients, 44 (21.8%) were found to have multiple MSAs. 63.6% of the 44 patients tested positive for anti-aminoacyl-tRNA synthetase antibodies (anti-ARS+) and 52.3% positive for anti-melanoma differentiation-associated protein-5 antibody (anti-MDA5+). The presence of multiple MSAs was associated with less rapidly progressive interstitial lung disease (RP-ILD), fever, rash, periungual erythema, more muscle involvement and dysphagia, higher albumin level, and higher positive rate of ANA antibody in anti-MDA5+ population. In anti-ARS+ population with multiple MSAs, there were more V-neck sign, skin ulcers, dysphagia and peripheral edema. No differences in survival rates were observed between patients with or without multiple MSAs in the overall and anti-ARS+ populations. However, the survival rate in anti-MDA5+ population with multiple MSAs was significantly higher than those without multiple MSAs (p = 0.003). Moreover, multiple MSAs remained an independent protective factor against mortality in multivariable Cox regression analysis of anti-MDA5+ population [HR 0.108 (95% CI 0.013, 0.908), p=0.041].
CONCLUSIONS:
Multiple MSAs coexist in some IIM patients and their existence indicates mixed features from concomitant MSAs in anti-MDA5+ population and anti-ARS+ population. Identifying multiple MSAs could help to discover a more favourable disease phenotype with decreased mortality in anti-MDA5+ population.