title: Suppressed signal transduction in the bronchial epithelium of patients with systemic sclerosis.
abstract: BACKGROUND
  Systemic sclerosis (SSc) is an autoimmune disorder, which frequently affects the lungs, with manifestations of interstitial lung disease (ILD) with lung fibrosis and of pulmonary hypertension. The pathogenesis remains largely unrecognised.
  
  
  OBJECTIVE
  The aim of this study was to elucidate the inflammation in the bronchial mucosa in patients with SSc.
  
  
  METHODS
  Twenty-three subjects diagnosed with SSc participated. Twelve of the SSc patients showed signs of ILD, four were smokers and seven were treated with oral corticosteroids. Seventeen non-smoking, age- and sex-matched healthy subjects served as controls. Bronchoscopy was performed to sample endobronchial mucosal biopsies, which were immunohistochemically stained using a panel of antibodies against inflammatory markers.
  
  
  RESULTS
  The number of neutrophils was significantly elevated in the submucosa of SSc patients, regardless of ILD, or whether the subject was smoking or using oral corticosteroids. No up-regulation of neutrophil chemoattractants or cytokines was seen in the bronchial epithelium. The signal transduction pathways and adhesion molecule expression tended to be suppressed or unchanged in SSc patients compared with controls.
  
  
  CONCLUSIONS
  It is concluded that SSc is associated with a chronic neutrophilic inflammation in the bronchial mucosal, with signs of suppressed signal transduction, regardless of the presence of interstitial lung disease.
authors.affiliation: Department of Respiratory Medicine and Allergy, University Hospital, 901 85 Umeå, Sweden. maria.sehlstedt@lung.umu.se
authors.forename: Maria
authors.initials: M
authors.lastname: Sehlstedt
authors.forename: Grethe Neumann
authors.initials: GN
authors.lastname: Andersen
authors.forename: Kenneth
authors.initials: K
authors.lastname: Nilsson
authors.forename: Anders
authors.initials: A
authors.lastname: Blomberg
authors.forename: Lucia
authors.initials: L
authors.lastname: Mincheva-Nilsson
authors.forename: Anders
authors.initials: A
authors.lastname: Waldenström
authors.forename: Solbritt
authors.initials: S
authors.lastname: Rantapää-Dahlqvist
authors.forename: Thomas
authors.initials: T
authors.lastname: Sandström
mesh_terms: D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology / Q000276:immunology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D008297:Male; D020780:Matrix Metalloproteinase 9 / Q000276:immunology; D008875:Middle Aged; D009504:Neutrophils / Q000166:cytology / Q000276:immunology*; D020545:Respiratory Mucosa / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D015398:Signal Transduction / Q000276:immunology*; D014057:Tomography, X-Ray Computed
chemical_list: D020780:Matrix Metalloproteinase 9