Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients

Rheumatology (Oxford). 2014 Apr;53(4):644-9. doi: 10.1093/rheumatology/ket380. Epub 2013 Dec 5.


Objectives: Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM outcome, and (iii) identify possible associations between pulmonary impairment and myositis-specific autoantibodies (MSAs).

Methods: Fifty-one JDM patients performed conventional spirometry in a cross-sectional follow-up study. The scores of the Myositis Damage Index (MDI), Myositis Damage by visual analogue scale (MYODAM-VAS) and physician's global damage assessment were used to estimate JDM outcome. ANAs, MSAs and myositis-associated autoantibodies were analysed in all patients.

Results: Forty-two patients (82%) (mean follow-up time 14.3 years) had normal lung function. Four patients (8%) were diagnosed with JDM-related restrictive interstitial lung disease. No patients reported pulmonary symptoms. Patients with restrictive pulmonary function had increased long-term damage estimated by MDI (P = 0.008), MYODAM-VAS (P = 0.04), global assessment (P = 0.03) and number of organ systems involved (P = 0.009). We found significant correlation between the restrictive pulmonary function test and damage by the MDI (r = 0.43, P = 0.003), MYODAM-VAS (r = 0.44, P = 0.002), and global damage assessment (r = 0.43, P = 0.003). No association was found between the restrictive pulmonary function test and autoantibodies.

Conclusion: In a long-term follow-up study of JDM patients, the majority of patients demonstrated normal lung function. However, restrictive pulmonary impairment was identified in 8% of patients, indicating a need for repetitive pulmonary follow-up in JDM patients. Restrictive pulmonary involvement was associated with increased long-term JDM damage.

Keywords: follow-up; juvenile dermatomyositis; myositis-associated autoantibodies; myositis-specific autoantibodies; pulmonary damage.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Antinuclear / immunology
  • Autoantibodies / immunology*
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cross-Sectional Studies
  • Dermatomyositis / complications
  • Dermatomyositis / immunology*
  • Dermatomyositis / physiopathology
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Forced Expiratory Volume
  • Humans
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / immunology*
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Ribonucleoproteins / immunology
  • Severity of Illness Index
  • Spirometry
  • Vital Capacity
  • Young Adult


  • Antibodies, Antinuclear
  • Autoantibodies
  • Jo-1 antibody
  • Mi-2 antibodies
  • Ribonucleoproteins
  • SS-A antigen

Supplementary concepts

  • Amyopathic dermatomyositis