title: Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease.
abstract: OBJECTIVE
  The aim of this paper is to assess the effect of calcineurin inhibitors (tacrolimus or cyclosporine) for treating patients with interstitial lung disease (ILD) associated with antisynthetase autoantibodies.
  
  
  METHODS
  Sixty patients with antisynthetase autoantibodies were identified in our myositis cohort of 179 patients. The medical records of 15 patients with antisynthetase autoantibody-associated ILD treated with tacrolimus/cyclosporine (11 for refractory disease and 4 as first-line therapy) between 1980 and 2011 were retrospectively reviewed. Serial pulmonary function tests were used to assess the clinical response. Qualitative data are presented as a number and percentage, and quantitative data as the median and interquartile range (IQR).
  
  
  RESULTS
  Patients were classified as having probable or definite idiopathic inflammatory myopathy (8 dermatomyositis and 4 polymyositis), and pure interstitial lung disease (3 cases). The 15 patients had received tacrolimus/cyclosporine for an average of 19 (IQR 14-30) months. Median age at onset of ILD was 42.3 (IQR 32.4-56.8) years and median duration of lung disease before administration of calcineurin inhibitors was 11 (IQR: 5-49) months. Median duration of follow-up was 24 (IQR 12-32) months. Thirteen patients had anti-histidyl-transfer RNA synthetase autoantibody (anti-Jo-1) and two had anti-alanyl-transfer RNA synthetase autoantibody (anti-PL-12). A more than 10% increase in FVC or stabilisation was observed in 13 (87%; 95%CI 56-98) patients who received calcineurin inhibitors (9 [81%] refractory cases and 4 [100%] as first-line therapy).
  
  
  CONCLUSIONS
  Calcineurin inhibitors seem to be a good therapeutic option for managing ILD associated with antisynthetase autoantibodies, not only in refractory cases, but also as first-line treatment.
authors: Department of Internal Medicine, Vall d´Hebron General Hospital. Universitat Autonoma de Barcelona, Spain. ane.labirua@gmail.com
authors: Ane
authors: A
authors: Labirua-Iturburu
authors: Albert
authors: A
authors: Selva-O'Callaghan
authors: Xavier
authors: X
authors: Martínez-Gómez
authors: Ernesto
authors: E
authors: Trallero-Araguás
authors: Moises
authors: M
authors: Labrador-Horrillo
authors: Miquel
authors: M
authors: Vilardell-Tarrés
mesh_terms: D000328:Adult; D000412:Alanine-tRNA Ligase / Q000276:immunology; D000974:Antibodies, Antinuclear / Q000276:immunology; D065095:Calcineurin Inhibitors; D015331:Cohort Studies; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000276:immunology; D012189:Retrospective Studies; D016559:Tacrolimus / Q000627:therapeutic use*; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D000974:Antibodies, Antinuclear; D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody; D016572:Cyclosporine; D000412:Alanine-tRNA Ligase; D016559:Tacrolimus