Distinct proteinase 3-induced cytokine patterns in Wegener´s granulomatosis, Churg-Strauss syndrome, and healthy controls

• Published in: Clinical and experimental rheumatology Vol. 29; no. 1 Suppl 64; pp. S57 - S62
• Main Authors: Fagin, Ursula, Csernok, Elena, Müller, Antje, Pitann, Silke, Fazio, Juliane, Krause, Kristina, Bremer, Philip, Wipfler-Freissmuth, Edith, Moosig, Frank, Gross, Wolfgang L, Lamprecht, Peter
• Format: Journal Article
• Language: English
• Published: Italy 01.01.2011
• Subjects: Adult; Aged; Aged, 80 and over; Antibodies, Antineutrophil Cytoplasmic - blood; Autoantigens; Case-Control Studies; Cell Line; Churg-Strauss Syndrome - enzymology; Churg-Strauss Syndrome - immunology; Cytokines - metabolism; Female; Flow Cytometry; Germany; Granulomatosis with Polyangiitis - enzymology; Granulomatosis with Polyangiitis - immunology; Humans; Inflammation Mediators - metabolism; Male; Middle Aged; Myeloblastin - immunology; T-Lymphocytes, Helper-Inducer - enzymology; T-Lymphocytes, Helper-Inducer - immunology; Th1 Cells - enzymology; Th1 Cells - immunology; Th17 Cells - enzymology; Th17 Cells - immunology; Th2 Cells - enzymology; Th2 Cells - immunology; Up-Regulation; Young Adult; Germany
• ISSN: 0392-856X

To analyse whether a specific cytokine pattern is elicited in response to the autoantigen proteinase 3 (PR3) in active Wegener's granulomatosis (WG). 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). 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. These data suggest that the cytokine response of the total CD4+ T-cell population and PR3-specific cells is influenced by the underlying disorder.