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FRI0419 The predictive prognostic factors for clinical course of polymyositis/dermatomyositis-associated interstitial lung disease
  1. Y Sugiyama1,
  2. R Yoshimi1,
  3. M Tamura1,
  4. N Hamada1,
  5. H Nagai1,
  6. N Tsuchida1,
  7. Y Soejima1,
  8. Y Kunishita1,
  9. D Kishimoto1,
  10. H Nakano1,
  11. R Kamiyama1,
  12. K Minegishi2,
  13. Y Asami1,
  14. Y Kirino1,
  15. S Ohno2,
  16. H Nakajima1,
  17. on behalf of Y-CURD Study Group
  1. 1Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medocine
  2. 2Center for Rheumatic Diseases, Yokohama City University Medical Center, Yokohama, Japan


Background Interstitial lung disease (ILD) and concomitant infectious diseases are the predominant causes of death in polymyositis/dermatomyositis (PM/DM). We have already reported that hypocapnea and ILD lesion in upper lung fields are independent prognostic factors. Micro RNA is a non-coding RNA, which has a certain function such as transcriptional regulation. miR-1 has been reported to be associated with myocyte differentiation and to decrease in muscle tissue from patients with inflammatory myopathies.

Objectives Here we investigated the association of serum miR-1 level with clinical course of PM/DM-associated ILD (PM/DM-ILD).

Methods We retrospectively analyzed clinical baseline, serum miR-1 level, initial therapeutic regimens, total amounts of PSL, clinical outcomes, and episode of infection of patient with PM/DM-ILD who had received initial treatment at six hospitals associated with Yokohama City University from 2003 to 2016. The serum miR-1 level was measured by quantitative real-time PCR.

Results One hundred sixteen (PM 22, DM 51, and clinically amyopathic DM 43) patients were included. The mean age was 56±15 years and 83 were female. As initial therapies, oral PSL, methylprednisolone (mPSL) pulse, intravenous cyclophosphamide (IVCY), and oral calcineurin inhibitor therapies were performed in 113 (97%), 80 (69%), 48 (41%) and 80 (69%), respectively. Forty-one patients had a serious infection at 51±38 days from initiation of immunosuppressants and 10 died of infections. Old age, low PaCO2 and albumin, high LDH and KL-6, high score of ILD, high initial dose of PSL, mPSL pulse, IVCY, calcineurin inhibitor and combination therapy were extracted as risk factors for infection by univariate analyses. A multivariate logistic regression analyses revealed that combination therapy (p=0.012, OR 2.83), old age (p=0.024, OR 2.12), high initial dose of PSL (p=0.024, OR 2.69), low albumin (p=0.031, OR 3.56), and low PaCO2 (p=0.038, OR 2.67) were independent risk factors for infection. Serum samples were obtained from total of 14 patients and 13 healthy controls. Serum miR-1 levels in PM/DM-ILD patients before treatment were significantly higher than those in healthy controls (p=0.047). Also serum miR-1 levels were significantly higher in PM/DM-ILD patients with concomitant infectious diseases as compared to patients without infectious diseases (p=0.043). We further divided the PM/DM-ILD cases into two groups by the serum miR-1 level. The higher miR-1 group showed poorer effectiveness of ILD treatment (p=0.040), and lower lymphocyte count (p=0.013) as compared to the lower miR-1 group.

Conclusions Appropriate monitoring is important for PM/DM-ILD, especially in older patients with malnutrition or decreased respiratory function. miR-1 can be a new biomarker for predicting treatment response and concomitant infectious diseases during treatment for PM/DM-ILD.


  1. Robert W. Georgantas et al, Inhibition of myogenic microRNAs 1, 133, and 206 by inflammatory cytokines links inflammation and muscle degeneration in adult inflammatory myopathies, Arthritis Rheum, 2014;66:1022–33.


Disclosure of Interest None declared

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