title: Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody.
abstract: OBJECTIVE
  The objective of this article was to establish the clinical course of interstitial lung disease (ILD) in scleroderma related to the presence of anti-PM/Scl antibody compared with anti-Scl-70 in a Spanish cohort. Furthermore, no study has thoroughly investigated the outcome of pulmonary function test in the first group of patients.
  
  
  METHODS
  A total of 63 Spanish patients with scleroderma and ILD were selected in a retrospective observational study. Among them, 14 were positive for anti-PM/Scl antibodies and 49 for anti-Scl-70. Clinical assessments, including pulmonary function test, were collected. Variations equal or greater than 10% in forced vital capacity (FVC) were considered significant. Progression-free survival of disease was defined as the period of stable illness since pulmonary fibrosis diagnosis.
  
  
  RESULTS
  Anti-Scl-70 patients had a higher frequency of diffuse SSc subset, peripheral vasculopathy, and gastrointestinal involvement. Inflammatory myopathy was associated to anti-PM/Scl antibody. Anti-PM/Scl patients presented more improvement in FVC during follow-up, 30.8% compared to a 7.1% in Scl-70 group (P = 0.04), with less worsening of this parameter (15.4% vs 52.4% in Scl-70 patients, P = 0.01), and secondary less frequency of severe restrictive pattern (FVC < 50%) (7.7% compared to 42.9% in the other group, P = 0.02). Regarding treatment, more anticalcineurinics were used in anti-PM/Scl patients, while cyclophosphamide and mycophenolate were mainly used in anti-Scl-70 patients. The progression-free survival of disease was higher in anti-PM/Scl patients, with 76% at 10 years from diagnosis of ILD against a 29% in the Scl-70 group.
  
  
  CONCLUSIONS
  Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile.
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain. Electronic address: alguille@vhebron.net.
authors.forename: Alfredo
authors.initials: A
authors.lastname: Guillen-Del Castillo
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Pilar Simeón-Aznar
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Vicent
authors.initials: V
authors.lastname: Fonollosa-Pla
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Serafín
authors.initials: S
authors.lastname: Alonso-Vila
authors.affiliation: Department of Trauma and Orthopaedic Surgery, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: María M
authors.initials: MM
authors.lastname: Reverte-Vinaixa
authors.affiliation: Department of Pneumology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Xavier
authors.initials: X
authors.lastname: Muñoz
authors.affiliation: Department of Radiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Esther
authors.initials: E
authors.lastname: Pallisa
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Albert
authors.initials: A
authors.lastname: Selva-O'allaghan
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Andreu
authors.initials: A
authors.lastname: Fernández-Codina
authors.affiliation: Department of Systemic Autoimmune Diseases, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Miquel
authors.initials: M
authors.lastname: Vilardell-Tarrés
mesh_terms: D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D015331:Cohort Studies; D004264:DNA Topoisomerases, Type I; D063326:Exosome Multienzyme Ribonuclease Complex / Q000276:immunology*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008168:Lung; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009687:Nuclear Proteins / Q000276:immunology; D011379:Prognosis; D016601:RNA-Binding Proteins / Q000276:immunology*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology* / Q000401:mortality; D015996:Survival Rate
chemical_list: D000888:Antibodies, Anti-Idiotypic; D015415:Biomarkers; C571398:EXOSC9 protein, human; D009687:Nuclear Proteins; D016601:RNA-Binding Proteins; C048100:Scl 70 antigen, human; D063326:Exosome Multienzyme Ribonuclease Complex; D004264:DNA Topoisomerases, Type I
keywords: Anti-PM/Scl antibodies; Anti-topoisomerase I antibodies; Interstitial lung disease; Prognosis; Pulmonary fibrosis; Pulmonary function test; Systemic sclerosis