PT  - JOURNAL ARTICLE
AU  - Li, Shanshan
AU  - Sun, Chao
AU  - Zhang, Ling
AU  - Han, Junfeng
AU  - Yang, Hanbo
AU  - Gao, Suhao
AU  - He, Linrong
AU  - Zhang, Peiyao
AU  - Lu, Xin
AU  - Shu, Xiaoming
AU  - Wang, Guochun
TI  - Clinical Heterogeneity of Patients With Antinuclear Matrix Protein 2 Antibody–Positive Myositis: A Retrospective Cohort Study in China
AID  - 10.3899/jrheum.211234
DP  - 2022 Aug 01
TA  - The Journal of Rheumatology
PG  - 922--928
VI  - 49
IP  - 8
4099  - http://www.jrheum.org/content/49/8/922.short
4100  - http://www.jrheum.org/content/49/8/922.full
SO  - J Rheumatol2022 Aug 01; 49
AB  - Objective Heterogeneity exists among patients with myositis who have antinuclear matrix protein 2 (anti-NXP2) antibodies, although they usually present with severe muscle weakness. This study aimed to investigate the differences in phenotypes and prognoses among adult patients with myositis who have anti-NXP2 antibodies.Methods Adult patients with myositis who have anti-NXP2 antibodies were enrolled from January 2010 to December 2019. Their clinical features and laboratory data were recorded retrospectively. We followed up on their survival status until June 30, 2020. A hierarchical cluster analysis, Kaplan-Meier curves, and classification and regression trees were used to analyze the data.Results A total of 70 adult patients with myositis who have anti-NXP2 antibodies were enrolled. All patients experienced muscle weakness. A total of 11 patients did not present with rashes during disease progression, and 43 patients developed dysphagia. In total, 21 patients had interstitial lung disease (ILD), whereas no patients had rapidly progressive ILD. Hierarchical cluster analysis identified 2 clusters. Patients in cluster 1 were younger at disease onset, had a higher incidence of subcutaneous calcification, and had a lower incidence of V sign and shawl sign. Patients in cluster 2 had a higher frequency of ILD, accompanied by lower levels of lymphocytes and higher levels of serum ferritin. Moreover, patients in cluster 2 had worse prognoses.Conclusion Patients with myositis who have anti-NXP2 antibodies may present with different phenotypes that are characterized by unique features and prognoses.