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Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with malignancy
  1. Yuki Ichimura1,
  2. Takashi Matsushita2,
  3. Yasuhito Hamaguchi2,
  4. Kenzo Kaji2,
  5. Minoru Hasegawa2,
  6. Yoshinori Tanino3,
  7. Yayoi Inokoshi3,
  8. Kazuhiro Kawai4,
  9. Takuro Kanekura4,
  10. Maria Habuchi5,
  11. Atsuyuki Igarashi5,
  12. Ryosuke Sogame6,
  13. Takashi Hashimoto6,
  14. Tomohiro Koga7,
  15. Ayako Nishino7,
  16. Naoko Ishiguro8,
  17. Naoki Sugimoto9,
  18. Rui Aoki10,
  19. Noriko Ando10,
  20. Tetsuya Abe11,
  21. Takashi Kanda11,
  22. Masataka Kuwana12,
  23. Kazuhiko Takehara2,
  24. Manabu Fujimoto2
  1. 1School of Medicine, Kanazawa University, Kanazawa, Japan
  2. 2Department of Dermatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
  3. 3Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan
  4. 4Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
  5. 5Division of Dermatology, Kanto Medical Center NTT EC, Tokyo, Japan
  6. 6Department of Dermatology, Kurume University School of Medicine, Kurume, Japan
  7. 7Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
  8. 8Department of Dermatology, Tokyo Women's Medical University, Tokyo, Japan
  9. 9Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan
  10. 10Department of Dermatology, Faculty of Medicine, University of Yamanashi, Yamanashi, Japan
  11. 11Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan
  12. 12Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
  1. Correspondence to Manabu Fujimoto, Department of Dermatology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa, Ishikawa 920-8641, Japan; fujimoto-m{at}


Objectives Myositis-specific autoantibodies (MSAs) are useful tools for identifying clinically homogeneous subsets and predicting prognosis of patients with idiopathic inflammatory myopathies (IIM) including polymyositis (PM) and dermatomyositis (DM). Recent studies have shown that anti-NXP2 antibody (Ab) is a major MSA in juvenile dermatomyositis (JDM). In this study the frequencies and clinical associations of anti-NXP2 Ab were evaluated in adult patients with IIM.

Methods Clinical data and serum samples were collected from 507 adult Japanese patients with IIM (445 with DM and 62 with PM). Eleven patients with JDM, 108 with systemic lupus erythematosus, 433 with systemic sclerosis and 124 with idiopathic pulmonary fibrosis were assessed as disease controls. Serum was examined for anti-NXP2 Ab by immunoprecipitation and western blotting using polyclonal anti-NXP2 Ab.

Results Seven patients (1.6%) with adult DM and one (1.6%) with adult PM were positive for anti-NXP2 Ab. Except for two patients with JDM, none of the disease controls were positive for this autoantibody. Among eight adult patients with IIM, three had internal malignancies within 3 years of diagnosis of IIM. Another patient with DM also had a metastatic cancer at the diagnosis. All of the carcinomas were at an advanced stage (stage IIIb–IV).

Conclusions While less common than in juvenile IIM, anti-NXP2 Ab was found in adult IIM. Anti-NXP2 Ab may be associated with adult IIM with malignancy.

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  • Ethic approval This study was conducted with the approval of Kanazawa University Graduate School of Medical Science and Kanazawa University Hospital.

  • Funding This work was supported by a grant of Research on Intractable Diseases from the Ministry of Health, Labour and Welfare of Japan.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.