Interstitial lung disease in anti-Jo-1 patients with antisynthetase syndrome

Arthritis Care Res (Hoboken). 2013 May;65(5):800-8. doi: 10.1002/acr.21895.


Objective: To assess the outcome of interstitial lung disease (ILD) in anti-Jo-1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti-Jo-1 patients with and without ILD.

Methods: Ninety-one anti-Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans.

Results: Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti-Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels.

Conclusion: Our findings confirm that ILD is a frequent complication in anti-Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti-Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antinuclear / blood*
  • Biomarkers / blood
  • Female
  • Follow-Up Studies
  • Humans
  • Lung Diseases, Interstitial / blood*
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / epidemiology*
  • Male
  • Middle Aged
  • Myositis / blood*
  • Myositis / diagnosis
  • Myositis / epidemiology*
  • Retrospective Studies


  • Antibodies, Antinuclear
  • Biomarkers
  • Jo-1 antibody

Supplementary concepts

  • Antisynthetase syndrome