title: Interstitial lung disease in anti-Jo-1 patients with antisynthetase syndrome.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Centre Hospitalier Universitaire Rouen and INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. isabelle.marie@chu-rouen.fr
authors.forename: I
authors.initials: I
authors.lastname: Marie
authors.forename: S
authors.initials: S
authors.lastname: Josse
authors.forename: P Y
authors.initials: PY
authors.lastname: Hatron
authors.forename: S
authors.initials: S
authors.lastname: Dominique
authors.forename: E
authors.initials: E
authors.lastname: Hachulla
authors.forename: A
authors.initials: A
authors.lastname: Janvresse
authors.forename: P
authors.initials: P
authors.lastname: Cherin
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
authors.forename: O
authors.initials: O
authors.lastname: Vittecoq
authors.forename: J-F
authors.initials: JF
authors.lastname: Menard
authors.forename: F
authors.initials: F
authors.lastname: Jouen
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology*; D012189:Retrospective Studies
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; C046750:Jo-1 antibody