Clinical Rheumatology

, Volume 37, Issue 6, pp 1573–1580 | Cite as

The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy

  • Ya-Mei Zhang
  • Han-Bo Yang
  • Jing-Li Shi
  • He Chen
  • Xiao-Ming Shu
  • Xin Lu
  • Guo-Chun Wang
  • Qing-Lin Peng
Original Article


Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. In our study, 388 IIM patients, 301 disease controls, and 167 healthy controls (HCs) were involved. An enzyme-linked immunosorbent assay (ELISA) was developed to detect serum anti-PUF60 levels and was validated using immunoblotting methods. Unpaired Mann-Whitney U test and Spearman correlation analysis were used when appropriate. Anti-PUF60 antibodies were observed in IIM patients at a frequency of 10.6% (41/388). Subgrouping analysis revealed that the prevalence of anti-PUF60 antibodies was 10% in DM, 5.5% in polymyositis (PM), 10% in immune-mediated necrotizing myositis (IMNM), and 26.5% in myositis-overlap syndrome. Anti-PUF60 antibodies were also observed in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren’s syndrome (SS) patients at a positive rate of 17.3, 14.5, and 10.1% respectively. Intriguingly, anti-PUF60 antibodies were frequently observed in clinically amyopathic dermatomyositis (CADM) patients and DM patients without currently known myositis autoantibodies. Furthermore, DM patients with anti-PUF60 antibodies had higher prevalence of skin ulcerations. Moreover, longitudinal investigation in eight DM patients with anti-PUF60 antibodies revealed that the antibodies levels decreased with disease remission. Anti-PUF60 antibodies were nonspecific for myositis, since they could be detected in other rheumatic diseases. Further investigation of anti-PUF60 antibodies may reveal shared pathogenic pathways in systemic autoimmune disorders.


Anti-PUF60 antibodies Clinically amyopathic dermatomyositis Disease activity Idiopathic inflammatory myopathies 



This work was supported by the National Natural Science Foundation of China (81701615, 91542121, 81571603), the Capital Foundation of Medical Developments (2016-2-4063), and the Science and Technology Commission Foundation of Beijing (Z151100004015143, Z171100001017208).

Compliance with ethical standards

This study was approved by the Ethics Committee of the Institutional Review Board at the China-Japan Friendship Hospital (reference number: 2016-117). All the participants provided written informed consent for the collection of samples and subsequent analysis.




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • Ya-Mei Zhang
    • 1
    • 2
  • Han-Bo Yang
    • 1
  • Jing-Li Shi
    • 1
  • He Chen
    • 1
  • Xiao-Ming Shu
    • 1
  • Xin Lu
    • 1
  • Guo-Chun Wang
    • 1
    • 2
  • Qing-Lin Peng
    • 1
  1. 1.Department of Rheumatology, Beijing Key Lab for Immune-Mediated Inflammatory DiseasesChina-Japan Friendship HospitalBeijingChina
  2. 2.Graduate School of Peking Union Medical CollegeBeijingChina

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