title: The pulmonary histopathology of anti-KS transfer RNA synthetase syndrome.
abstract: BACKGROUND
  The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described.
  
  
  OBJECTIVE
  To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome.
  
  
  METHODS
  We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies.
  
  
  RESULTS
  The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia.
  
  
  CONCLUSIONS
  Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.
authors.affiliation: From the Departments of Pathology (Drs Schneider and Yousem, Mr Bi), Rheumatology (Drs Aggarwal and Oddis), and Pulmonary Medicine (Dr Gibson), UPMC Presbyterian, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
authors.forename: Frank
authors.initials: F
authors.lastname: Schneider
authors.forename: Rohit
authors.initials: R
authors.lastname: Aggarwal
authors.forename: David
authors.initials: D
authors.lastname: Bi
authors.forename: Kevin
authors.initials: K
authors.lastname: Gibson
authors.forename: Chester
authors.initials: C
authors.lastname: Oddis
authors.forename: Samuel A
authors.initials: SA
authors.lastname: Yousem
mesh_terms: D000328:Adult; D000604:Amino Acyl-tRNA Synthetases / Q000276:immunology*; D001226:Aspartate-tRNA Ligase / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008168:Lung / Q000276:immunology / Q000473:pathology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000175:diagnosis* / Q000276:immunology; D012346:RNA, Transfer, Amino Acyl / Q000276:immunology; D012042:Registries; D012189:Retrospective Studies; D012196:Review Literature as Topic
chemical_list: D001323:Autoantibodies; D012346:RNA, Transfer, Amino Acyl; D000604:Amino Acyl-tRNA Synthetases; D001226:Aspartate-tRNA Ligase; C029194:asparaginyl-tRNA synthetase