title: Distinct proteinase 3-induced cytokine patterns in Wegener´s granulomatosis, Churg-Strauss syndrome, and healthy controls.
abstract: OBJECTIVE
  To analyse whether a specific cytokine pattern is elicited in response to the autoantigen proteinase 3 (PR3) in active Wegener's granulomatosis (WG).
  
  
  METHODS
  Six-colour flow cytometry was used to analyse cytokine production and surface markers of the total CD4+ T-cell population ex vivo and in PR3-stimulated T-cell lines of patients with active PR3-ANCA-positive WG, PR3-ANCA-negative Churg-Strauss syndrome (CSS), and healthy controls (HC).
  
  
  RESULTS
  The cytokine response of the total PB CD4+ T cell population was skewed towards distinct pro-inflammatory cytokine patterns in WG (Th1-type) and CSS (Th17, Th1-/Th2-type). Th2-type as well as Th17 cell populations including Th17/Th1, Th17/Th2 and Th22 cells were elicited in response to PR3 stimulation in WG. In contrast, CSS patients displayed a Th2-type dominated response following PR3 stimulation.
  
  
  CONCLUSIONS
  These data suggest that the cytokine response of the total CD4+ T-cell population and PR3-specific cells is influenced by the underlying disorder.
authors.affiliation: University of Lubeck, Department of Rheumatology, Vasculitis Center UKSH & Clinical Center Bad Bramstedt, Lubeck, Germany. ursula.fagin@uk-sh.de
authors.forename: Ursula
authors.initials: U
authors.lastname: Fagin
authors.forename: Elena
authors.initials: E
authors.lastname: Csernok
authors.forename: Antje
authors.initials: A
authors.lastname: Müller
authors.forename: Silke
authors.initials: S
authors.lastname: Pitann
authors.forename: Juliane
authors.initials: J
authors.lastname: Fazio
authors.forename: Kristina
authors.initials: K
authors.lastname: Krause
authors.forename: Philip
authors.initials: P
authors.lastname: Bremer
authors.forename: Edith
authors.initials: E
authors.lastname: Wipfler-Freissmuth
authors.forename: Frank
authors.initials: F
authors.lastname: Moosig
authors.forename: Wolfgang L
authors.initials: WL
authors.lastname: Gross
authors.forename: Peter
authors.initials: P
authors.lastname: Lamprecht
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001324:Autoantigens; D016022:Case-Control Studies; D002460:Cell Line; D015267:Churg-Strauss Syndrome / Q000201:enzymology / Q000276:immunology*; D016207:Cytokines / Q000378:metabolism*; D005260:Female; D005434:Flow Cytometry; D005858:Germany; D014890:Granulomatosis with Polyangiitis / Q000201:enzymology / Q000276:immunology*; D006801:Humans; D018836:Inflammation Mediators / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology*; D006377:T-Lymphocytes, Helper-Inducer / Q000201:enzymology / Q000276:immunology*; D018417:Th1 Cells / Q000201:enzymology / Q000276:immunology; D058504:Th17 Cells / Q000201:enzymology / Q000276:immunology; D018418:Th2 Cells / Q000201:enzymology / Q000276:immunology; D015854:Up-Regulation; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001324:Autoantigens; D016207:Cytokines; D018836:Inflammation Mediators; D053146:Myeloblastin