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Research ArticleMyositis

Clinical Heterogeneity of Patients With Antinuclear Matrix Protein 2 Antibody–Positive Myositis: A Retrospective Cohort Study in China

Shanshan Li, Chao Sun, Ling Zhang, Junfeng Han, Hanbo Yang, Suhao Gao, Linrong He, Peiyao Zhang, Xin Lu, Xiaoming Shu and Guochun Wang
The Journal of Rheumatology August 2022, 49 (8) 922-928; DOI: https://doi.org/10.3899/jrheum.211234
Shanshan Li
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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Chao Sun
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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Ling Zhang
2L. Zhang, MD, P. Zhang, MD, Department of Radiology, China-Japan Friendship Hospital;
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Junfeng Han
3J. Han, PhD, Key Laboratory of Advanced Optoelectronic Quantum Architecture and Measurement, Ministry of Education, School of Physics, Beijing Institute of Technology;
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Hanbo Yang
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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Suhao Gao
4S. Gao, MSc, School of Statistics, Renmin University of China, Beijing, China
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Linrong He
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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Peiyao Zhang
2L. Zhang, MD, P. Zhang, MD, Department of Radiology, China-Japan Friendship Hospital;
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Xin Lu
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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Xiaoming Shu
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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  • ORCID record for Xiaoming Shu
  • For correspondence: sxm992283@hotmail.com guochunwang@hotmail.com
Guochun Wang
1S. Li, MD, C. Sun, MM, H. Yang, MD, L. He, MD, X. Lu, MD, X. Shu, MD, G. Wang, MD, Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital;
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    Figure 1.

    The hierarchical cluster analysis of patients with anti-NXP2 antibody–positive myositis. The dendrogram was generated using Euclidean distance and the Ward agglomerative method. The bold vertical line indicates the height of fusion into the proposed clusters, and the X-axis indicates the individuals (n = 70) at the bottom of the dendrogram. anti-NXP2: antinuclear matrix protein 2.

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    Figure 2.

    The comparison of survival time between cluster 1 and cluster 2. Cum: cumulative; m: months.

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    Figure 3.

    The classification and regression trees of each cluster. The regression tree model was used to identify the class within which a target variable would presumably fall. Using classification and regression trees, age of disease onset was identified as a predictor that positioned patients into different clusters. The main clinical characteristics of the different clusters are shown in the lower boxes. anti-NXP2: antinuclear matrix protein 2.

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    Table 1.

    Comparison of clinical features between patients in cluster 1 and cluster 2.

     Cluster 1, n = 37Cluster 2, n = 33P
    Age at onset, yrs, mean (SD)30.41 (7.83)52.03 (9.76)< 0.001**
    Age at onset, yrs
            18-3020 (54)0 (0)< 0.001**
            31-4014 (38)3 (9)0.005**
            41-503 (8)11 (33)0.008
            51-600 (0)13 (39)< 0.001**
            61-700 (0)4 (12)0.045
            > 700 (0)2 (6)0.22
    Sex  0.40
            Male16 (43)11 (33) 
            Female21 (57)22 (67) 
    Duration from onset to diagnosis, months, median (IQR)2.0 (1.5-5.0)2.0 (1.0-3.5)0.16
    Muscle weakness  0.27
            Any muscle weakness37 (100)33 (100) 
            Severe muscle weakness10 (27)13 (39) 
    Myalgia29 (78)25 (76)0.79
    Rash
            All rash types29 (78)30 (91)0.15
            Heliotrope rash23 (62)22 (67)0.70
            V sign9 (24)22 (67)< 0.001**
            Shawl sign4 (11)17 (52)< 0.001**
            Holster sign4 (11)4 (11)1.000
            Mechanic’s hands3 (8)7 (19)0.22
            Gottron sign and papules8 (22)12 (32)0.17
            Cutaneous ulcer3 (8)6 (16)0.29
            Subcutaneous calcification8 (22)1 (3)0.03*
            Subcutaneous edema12 (33)14 (42)0.39
    Arthritis2 (5)3 (9)0.66
    Interstitial lung disease6 (16)15 (45)0.008**
    Dysphagia23 (62)20 (61)0.89
    WBCs, mean (SD)a
            All WBCs, × 109/L8.12 (3.91)9.23 (4.33)0.26
            Neutrophils, × 109/L6.24 (3.81)7.61 (4.06)0.15
            Lymphocytes
                All lymphocytes, × 109/L1.23 (0.67)0.97 (0.36)0.04*
                CD3, cells/μL850.65 (515.96)741.35 (365.80)0.33
                CD4, cells/μL557.29 (344.89)511.52 (280.31)0.56
                CD8, cells/μL273.85 (213.90)212.77 (113.26)0.15
                NK, cells/μL, median (IQR)31.00 (19.50-64.25)46.50 (28.25-80.75)0.08
                B1 (cells/μL)63.65 (74.71)46.97 (64.53)0.35
                B2 (cells/μL)248.85 (180.29)244.76 (32.65)0.94
    ALT, U/L, median (IQR)48.0 (25.5-92.0)67.0 (38.0-157.5)0.06
    AST, U/L, median (IQR)45.0 (22.0-107.5)132.0 (38.5-271.0)0.02*
    CK, U/L, median (IQR)233.0 (95.5-1685.0)1462.0 (177.0-6163.0)0.01*
    LDH, U/L, mean (SD)383.65 (238.32)590.73 (370.32)0.006**
    Albumin, g/L, mean (SD)36.69 (5.29)33.19 (7.23)0.02*
    Prealbumin, mg/L, mean (SD)220.10 (98.05)182.72 (74.67)0.08
    C3, mg/dL, mean (SD)75.24 (25.79)86.32 (17.93)0.04*
    C4, mg/dL, mean (SD)16.94 (6.43)20.16 (4.95)0.02*
    CRP, mg/dL, median (IQR)0.44 (0.19-0.94)0.63 (0.39-1.27)0.12
    ESR, mm/h, median (IQR)11.0 (7.0-21.5)16.0 (8.0-33.0)0.16
    Serum ferritin, ng/mL, median (IQR)139.2 (54.0-606.0)312.9 (145.4-768.5)0.01*
    ANA positive13 (35)17 (52)0.17
    Cancer-associated myositis1 (3)3 (12)0.34b
    Muscle biopsy30 (81)25 (76)0.59
    • Data are in n (%) unless otherwise indicated.

    • ↵aAll WBC values are reported as mean (SD), except for NK, which is reported as median (IQR).

    • ↵bThese data were deleted because patients were lost to follow-up.

    • ↵*P < 0.05.

    • ↵**P < 0.01. ALT: alanine aminotransferase; ANA: antinuclear antibody; AST: aspartate aminotransferase; CK: creatine kinase; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; LDH: lactate dehydrogenase ; NK: natural killer; WBC: white blood cell.

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Vol. 49, Issue 8
1 Aug 2022
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Clinical Heterogeneity of Patients With Antinuclear Matrix Protein 2 Antibody–Positive Myositis: A Retrospective Cohort Study in China
Shanshan Li, Chao Sun, Ling Zhang, Junfeng Han, Hanbo Yang, Suhao Gao, Linrong He, Peiyao Zhang, Xin Lu, Xiaoming Shu, Guochun Wang
The Journal of Rheumatology Aug 2022, 49 (8) 922-928; DOI: 10.3899/jrheum.211234

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Clinical Heterogeneity of Patients With Antinuclear Matrix Protein 2 Antibody–Positive Myositis: A Retrospective Cohort Study in China
Shanshan Li, Chao Sun, Ling Zhang, Junfeng Han, Hanbo Yang, Suhao Gao, Linrong He, Peiyao Zhang, Xin Lu, Xiaoming Shu, Guochun Wang
The Journal of Rheumatology Aug 2022, 49 (8) 922-928; DOI: 10.3899/jrheum.211234
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Keywords

AUTOANTIBODIES
antinuclear matrix protein 2 antibody
DERMATOMYOSITIS
PHENOTYPE
PROGNOSIS

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