title: Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: heterogeneity within the syndrome.
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.
  
  
  CONCLUSIONS
  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."
authors.affiliation: Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hamaguchi
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Kenzo
authors.initials: K
authors.lastname: Kaji
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Komura
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Masanari
authors.initials: M
authors.lastname: Kodera
authors.forename: Eiji
authors.initials: E
authors.lastname: Muroi
authors.forename: Keita
authors.initials: K
authors.lastname: Fujikawa
authors.forename: Mariko
authors.initials: M
authors.lastname: Seishima
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Yamada
authors.forename: Ryo
authors.initials: R
authors.lastname: Yamada
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Masataka
authors.initials: M
authors.lastname: Kuwana
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000604:Amino Acyl-tRNA Synthetases / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D003882:Dermatomyositis / Q000097:blood / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000453:epidemiology / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000453:epidemiology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000097:blood / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000097:blood / Q000453:epidemiology / Q000276:immunology; D013577:Syndrome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D000604:Amino Acyl-tRNA Synthetases