Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
10.3760/cma.j.cn112140-20250730-00689
- VernacularTitle:多中心抗NXP2抗体阳性幼年皮肌炎临床特征及重症危险因素分析
- Author:
Huiyuan YANG
1
;
Wanzhen GUAN
;
Ling2 YANG
;
Haimei LIU
;
Xiaoqing3 LI
;
Haiguo YU
;
Meiping LU
;
Jun YANG
;
Xiaohui LIU
;
Hongxia ZHANG
;
Wei ZHANG
;
Jihong XIAO
;
Xiaozhong LI
;
Guomin LI
;
Hong CHANG
;
Sheng HAO
;
Yue DU
;
Daliang XU
;
Ling WU
;
Wenjie ZHENG
;
Li LIU
;
Xinhui JIANG
;
Shaohui ZHU
;
Dongmei ZHAO
;
Xuemei TANG
;
Li SUN
Author Information
1. 国家儿童医学中心 复旦大学附属儿科医院风湿科，上海 201102
- Publication Type:Journal Article
- Keywords:
Dermatomyositis;
Anti-NXP2 antibody;
Risk factors
- From:
Chinese Journal of Pediatrics
2025;63(12):1299-1305
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.
