title: IgG4 immune response in Churg-Strauss syndrome.
abstract: OBJECTIVE
  T-helper type 2 responses are crucial in Churg-Strauss syndrome (CSS) and may enhance the production of IgG4 antibodies. The authors assessed the IgG4 immune response in CSS patients.
  
  
  METHODS
  The authors included 46 consecutive patients with CSS (24 with active and 22 with quiescent disease), 26 with granulomatosis with polyangiitis (GPA, Wegener's), 25 with atopic asthma and 20 healthy controls and determined serum IgG, IgM, IgA, IgE and IgG subclass levels. Tissue infiltration by IgG4 plasma cells was assessed in nine patients with CSS, 10 with GPA, 22 with chronic sinusitis (11 with and 11 without eosinophilia).
  
  
  RESULTS
  IgG4 levels were markedly higher in active CSS patients than in controls (p<0.001 vs all control groups). Serum IgG4 correlated with the number of disease manifestations (r=0.52, p=0.01) and the Birmingham vasculitis activity score (r=0.64, p=0.001). Longitudinal analysis in 12 CSS cases showed that both the IgG4 level and IgG4/IgG ratio dropped during disease remission (p=3×10(-5) and p=6×10(-4), respectively). Tissue analysis did not show an increased IgG4 plasma cell infiltration in CSS biopsies compared with control groups.
  
  
  CONCLUSIONS
  Serum IgG4 levels are markedly elevated in active CSS and correlate with the number of organ manifestations and disease activity.
authors: Department of Clinical Medicine, Nephrology and Health Sciences, University of Parma, Parma, Italy.
authors: Augusto
authors: A
authors: Vaglio
authors: Johanna D
authors: JD
authors: Strehl
authors: Bernhard
authors: B
authors: Manger
authors: Federica
authors: F
authors: Maritati
authors: Federico
authors: F
authors: Alberici
authors: Christian
authors: C
authors: Beyer
authors: Jürgen
authors: J
authors: Rech
authors: Renato A
authors: RA
authors: Sinico
authors: Francesco
authors: F
authors: Bonatti
authors: Luisita
authors: L
authors: Battistelli
authors: Jörg H W
authors: JH
authors: Distler
authors: Georg
authors: G
authors: Schett
authors: Jochen
authors: J
authors: Zwerina
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001249:Asthma / Q000276:immunology; D016022:Case-Control Studies; D015267:Churg-Strauss Syndrome / Q000276:immunology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007136:Immunoglobulins / Q000097:blood; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G; D007136:Immunoglobulins