RT Journal Article
SR Electronic
T1 IgG4 immune response in Churg–Strauss syndrome
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 390
OP 393
DO 10.1136/ard.2011.155382
VO 71
IS 3
A1 Augusto Vaglio
A1 Johanna D Strehl
A1 Bernhard Manger
A1 Federica Maritati
A1 Federico Alberici
A1 Christian Beyer
A1 Jürgen Rech
A1 Renato A Sinico
A1 Francesco Bonatti
A1 Luisita Battistelli
A1 Jörg H W Distler
A1 Georg Schett
A1 Jochen Zwerina
YR 2012
UL http://ard.bmj.com/content/71/3/390.abstract
AB 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.