Antisynthetase syndrome: Pulmonary computed tomography findings of adult patients with antibodies to aminoacyl-tRNA synthetases

Eur J Radiol. 2016 Aug;85(8):1421-6. doi: 10.1016/j.ejrad.2016.05.012. Epub 2016 May 24.

Abstract

Objectives: To describe the pulmonary CT findings in patients with anti-ARS-antibody-positive interstitial lung disease (anti-ARS-ILD) METHODS: The CT findings of 64 patients with anti-ARS-ILD were retrospectively reviewed. The images were retrospectively reviewed independently by 2 chest radiologists, and the final decision on the CT findings was made by a third chest radiologist.

Results: There were 16 male and 48 female patients, aged 54.2±13.4 years. Sixteen patients had anti Jo-1, 24 had anti-EJ, 9 had anti-PL-7, 7 had anti-PL-12, 5 had anti-KS, and 3 had anti-OJ antibodies. Overall, 63 patients (98.4%) had CT findings predominantly in the lower lobe; 61 patients (95.3%) showed peripheral opacities, and 47 patients (73.4%) showed peribronchovascular opacities. Ground-glass attenuation, consolidation, and reticulation showed similar distribution patterns. Regarding detailed CT findings, 89.1% of patients had lower volume loss, 76.6% had interlobular septal thickening, and 67.2% had thickening of bronchovascular bundles. The final radiologic diagnoses were as follows: inconsistent with usual interstitial pneumonia (UIP) in 63 patients (98.4%), which included nonspecific interstitial pneumonia (NSIP) in 35 patients (55.6%), organizing pneumonia (OP) in 4 patients (6.3%), and OP with fibrosis in 22 patients (34.9%).

Conclusions: The characteristic CT findings of patients with anti-ARS-ILD were areas of ground-glass attenuation and reticulation, predominantly distributed as lower and peribronchovascular lesions, which is compatible with NSIP. One-third of patients showed OP with fibrosis.

Keywords: Aminoacyl-tRNA synthetases; Antisynthetase syndrome; Dermatomyositis; Interstitial lung disease; Polymyositis.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Amino Acyl-tRNA Synthetases / blood
  • Amino Acyl-tRNA Synthetases / immunology*
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Female
  • Humans
  • Lung / diagnostic imaging
  • Lung / immunology
  • Male
  • Middle Aged
  • Myositis / blood
  • Myositis / diagnostic imaging*
  • Myositis / immunology*
  • Retrospective Studies
  • Tomography, X-Ray Computed*
  • Young Adult

Substances

  • Autoantibodies
  • Amino Acyl-tRNA Synthetases

Supplementary concepts

  • Antisynthetase syndrome