Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: heterogeneity within the syndrome

PLoS One. 2013;8(4):e60442. doi: 10.1371/journal.pone.0060442. Epub 2013 Apr 3.

Abstract

Objective: To identify similarities and differences in the clinical features of adult Japanese patients with individual anti-aminoacyl-tRNA synthetase antibodies (anti-ARS Abs).

Methods: This was a retrospective analysis of 166 adult Japanese patients with anti-ARS Abs detected by immunoprecipitation assays. These patients had visited Kanazawa University Hospital or collaborating medical centers from 2003 to 2009.

Results: Anti-ARS Ab specificity included anti-Jo-1 (36%), anti-EJ (23%), anti-PL-7 (18%), anti-PL-12 (11%), anti-KS (8%), and anti-OJ (5%). These anti-ARS Abs were mutually exclusive, except for one serum Ab that had both anti-PL-7 and PL-12 reactivity. Myositis was closely associated with anti-Jo-1, anti-EJ, and anti-PL-7, while interstitial lung disease (ILD) was correlated with all 6 anti-ARS Abs. Dermatomyositis (DM)-specific skin manifestations (heliotrope rash and Gottron's sign) were frequently observed in patients with anti-Jo-1, anti-EJ, anti-PL-7, and anti-PL-12. Therefore, most clinical diagnoses were polymyositis or DM for anti-Jo-1, anti-EJ, and anti-PL-7; clinically amyopathic DM or ILD for anti-PL-12; and ILD for anti-KS and anti-OJ. Patients with anti-Jo-1, anti-EJ, and anti-PL-7 developed myositis later if they had ILD alone at the time of disease onset, and most patients with anti-ARS Abs eventually developed ILD if they did not have ILD at disease onset.

Conclusion: Patients with anti-ARS Abs are relatively homogeneous. However, the distribution and timing of myositis, ILD, and rashes differ among patients with individual anti-ARS Abs. Thus, identification of individual anti-ARS Abs is beneficial to define this rather homogeneous subset and to predict clinical outcomes within the "anti-synthetase syndrome."

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Amino Acyl-tRNA Synthetases / immunology*
  • Autoantibodies / blood*
  • Dermatomyositis / blood
  • Dermatomyositis / drug therapy
  • Dermatomyositis / epidemiology
  • Dermatomyositis / immunology*
  • Female
  • Humans
  • Lung Diseases, Interstitial / blood
  • Lung Diseases, Interstitial / epidemiology
  • Lung Diseases, Interstitial / immunology
  • Lupus Erythematosus, Systemic / blood
  • Lupus Erythematosus, Systemic / epidemiology
  • Lupus Erythematosus, Systemic / immunology
  • Male
  • Middle Aged
  • Polymyositis / blood
  • Polymyositis / drug therapy
  • Polymyositis / epidemiology
  • Polymyositis / immunology*
  • Prevalence
  • Scleroderma, Systemic / blood
  • Scleroderma, Systemic / epidemiology
  • Scleroderma, Systemic / immunology
  • Syndrome
  • Young Adult

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Amino Acyl-tRNA Synthetases

Supplementary concepts

  • Amyopathic dermatomyositis

Grant support

This work was supported by a research grant on intractable diseases from the Ministry of Health, Labor and Welfare of Japan (to Manabu Fujimoto). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.