title: Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome.
abstract: BACKGROUND
  The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS).
  
  
  METHODS
  The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection.
  
  
  RESULTS
  Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP).
  
  
  CONCLUSIONS
  Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan.
authors: Department of Internal Medicine, CHU Rouen, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. isabelle.marie@chu-rouen.fr
authors: I
authors: I
authors: Marie
authors: S
authors: S
authors: Josse
authors: O
authors: O
authors: Decaux
authors: E
authors: E
authors: Diot
authors: C
authors: C
authors: Landron
authors: P
authors: P
authors: Roblot
authors: S
authors: S
authors: Jouneau
authors: P Y
authors: PY
authors: Hatron
authors: E
authors: E
authors: Hachulla
authors: O
authors: O
authors: Vittecoq
authors: J-F
authors: JF
authors: Menard
authors: F
authors: F
authors: Jouen
authors: S
authors: S
authors: Dominique
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D003882:Dermatomyositis / Q000276:immunology / Q000401:mortality; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000276:immunology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000276:immunology* / Q000401:mortality; D017285:Polymyositis / Q000276:immunology / Q000401:mortality; D012189:Retrospective Studies; D013914:Threonine-tRNA Ligase / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D013914:Threonine-tRNA Ligase