IgG4 immune response in Churg-Strauss syndrome

Vaglio A, Strehl JD, Manger B, Maritati F, Alberici F, Beyer C, Rech J, Rech J, Sinico RA, Bonatti F, Battistelli L, Distler J, Schett G, Zwerina J (2012)

Publication Type: Journal article

Publication year: 2012

Journal

Annals of the Rheumatic Diseases BMJ Publishing Group

Book Volume: 71

Pages Range: 390-393

Journal Issue: 3

DOI: 10.1136/ard.2011.155382

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 with CRIS profile

Bernhard Manger Professur für Rheumatologie und Klinische Immunologie Christian Beyer Department of Medicine 3 – Rheumatology and Immunology Hans Jürgen Rech Department of Medicine 3 – Rheumatology and Immunology Jörg Distler Professur für Molekulare Mechanismen der Organfibrose Georg Schett Lehrstuhl für Innere Medizin III

Involved external institutions

University of Parma / Università degli Studi di Parma IT Italy (IT) Azienda Ospedaliero-Universitaria di Parma / Parma University Hospital IT Italy (IT) Ospedale San Carlo Borromeo IT Italy (IT)

How to cite

APA:

Vaglio, A., Strehl, J.D., Manger, B., Maritati, F., Alberici, F., Beyer, C.,... Zwerina, J. (2012). IgG4 immune response in Churg-Strauss syndrome. Annals of the Rheumatic Diseases, 71(3), 390-393. https://doi.org/10.1136/ard.2011.155382

MLA:

Vaglio, Augusto, et al. "IgG4 immune response in Churg-Strauss syndrome." Annals of the Rheumatic Diseases 71.3 (2012): 390-393.

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