Cytokine profiles in polymyositis and dermatomyositis complicated by rapidly progressive or chronic interstitial lung disease

Rheumatology (Oxford). 2014 Dec;53(12):2196-203. doi: 10.1093/rheumatology/keu258. Epub 2014 Jun 26.


Objective: PM and DM are often complicated by interstitial lung disease (ILD). In this study we aimed to evaluate various serum cytokines in patients with PM/DM with ILD so as to clarify the differences in pathophysiology between anti-melanoma differentiation-associated gene 5 antibody-associated ILD (anti-MDA5-ILD) and anti-aminoacyl tRNA synthetase antibody-associated ILD (anti-ARS-ILD).

Methods: We evaluated the serum cytokine profiles of 38 patients with PM/DM and compared the cytokine profiles of the non-ILD and ILD subsets as well as the anti-MDA5-ILD and anti-ARS-ILD subsets.

Results: The myositis intention-to-treat activity index score, which indicates whole disease activity, significantly correlated with serum IL-6, IL-8, TNF-α and IP-10. These cytokine levels were significantly higher in the ILD subset than the non-ILD subset and were lower in the ILD subset following treatment. By multivariate analysis, TNF-α was the most significant cytokine [P = 0.0006, odds ratio (OR) 1.4, CI 1.1, 2.2] associated with PM/DM with ILD. IL-8 levels were significantly higher in anti-MDA5-ILD than in anti-ARS-ILD, although IL-6, TNF-α and IP-10 levels were high in both subsets. IL-8 was the most significant cytokine (P = 0.0006, OR 1.5, CI 1.1, 3.0) associated with anti-MDA5-ILD by multivariate analysis. Moreover, the ratio of IL-4 to IFN-γ was lower in anti-MDA5-ILD than in anti-ARS-ILD.

Conclusion: IL-6, IL-8, TNF-α and IP-10 are associated with global disease activity in PM/DM. These cytokine levels were high, especially in the ILD subset. Serum IL-8 levels and the balance between IL-4 and IFN-γ may contribute to the differences in pathophysiology between anti-ARS-ILD and anti-MDA5-ILD.

Keywords: IL-6; IL-8; TNF-α; dermatomyositis; interstitial lung disease; polymyositis.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Chronic Disease
  • Cytokines / blood*
  • Dermatomyositis / complications
  • Dermatomyositis / immunology*
  • Disease Progression
  • Female
  • Humans
  • Interleukin-6 / blood
  • Interleukin-8 / blood
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / immunology*
  • Male
  • Middle Aged
  • Polymyositis / complications
  • Polymyositis / immunology*
  • Prognosis
  • Retrospective Studies
  • Severity of Illness Index
  • Tumor Necrosis Factor-alpha / blood


  • Cytokines
  • IL6 protein, human
  • Interleukin-6
  • Interleukin-8
  • Tumor Necrosis Factor-alpha