Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis

Rheumatology (Oxford). 2015 May;54(5):784-91. doi: 10.1093/rheumatology/keu385. Epub 2014 Oct 6.


Objective: Rapidly progressive interstitial lung disease (RP-ILD) is a rare but potentially fatal complication of JDM. The aim of this study was to establish markers for the prediction and early diagnosis of RP-ILD associated with JDM.

Methods: The clinical records of 54 patients with JDM were retrospectively reviewed: 10 had RP-ILD (7 died, 3 survived), 19 had chronic ILD and 24 were without ILD. Routine tests included a high-resolution CT (HRCT) scan of the chest and measurement of serum levels of creatine phosphokinase, ferritin and Krebs von den Lungen-6 (KL-6). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies and IL-18 levels were measured by ELISA.

Results: No differences were found in the ratio of juvenile clinically amyopathic DM between the three groups. Initial chest HRCT scan findings were variable and could not distinguish between RP-ILD and chronic ILD. Anti-MDA5 antibodies were positive in all 8 patients with RP-ILD and 10 of 14 with chronic ILD, but none of the patients without ILD. Serum levels of anti-MDA5 antibody, ferritin, KL-6 and IL-18 were significantly higher in the RP-ILD group than in the chronic ILD and non-ILD groups. Serum levels of IL-18 positively correlated with serum KL-6 (R = 0.66, P < 0.001).

Conclusion: High serum levels of IL-18, KL-6, ferritin and anti-MDA5 antibodies (e.g. >200 units by ELISA) are associated with RP-ILD. These can be used as an indication for early intensive treatment. Both alveolar macrophages and autoimmunity to MDA5 are possibly involved in the development of RP-ILD associated with JDM.

Keywords: KL-6; anti-melanoma differentiation-associated gene 5; ferritin; interleukin-18; interstitial lung disease; juvenile dermatomyositis.

Publication types

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

MeSH terms

  • Adolescent
  • Antibodies, Anti-Idiotypic / blood
  • Biomarkers / blood
  • Child
  • Child, Preschool
  • DEAD-box RNA Helicases / immunology
  • Dermatomyositis / blood*
  • Dermatomyositis / complications*
  • Dermatomyositis / ethnology
  • Disease Progression*
  • Female
  • Ferritins / blood
  • Humans
  • Infant
  • Interferon-Induced Helicase, IFIH1
  • Interleukin-18 / blood
  • Japan
  • Lung Diseases, Interstitial / blood*
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / mortality
  • Male
  • Mucin-1 / blood
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies


  • Antibodies, Anti-Idiotypic
  • Biomarkers
  • Interleukin-18
  • MUC1 protein, human
  • Mucin-1
  • Ferritins
  • IFIH1 protein, human
  • DEAD-box RNA Helicases
  • Interferon-Induced Helicase, IFIH1

Supplementary concepts

  • Amyopathic dermatomyositis