Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

IgG4 Immune Response in Churg-Strauss Syndrome.

Vaglio1,  Augusto, Strehl2,  Johanna, Manger2,  Bernhard, Maritati1,  Federica, Alberici1,  Federico, Beyer2,  Christian, Rech2,  Juergen

University of Parma, Parma, Italy
University of Erlangen-Nuremberg, Erlangen, Germany
Ospedale San Carlo Borromeo, Milano, Italy


Churg-Strauss Syndrome (CSS) is a systemic vasculitis usually occurring in individuals with a history of asthma. TH2 responses are common in allergic diseases and enhance the production of immunoglobulin (Ig)G4 antibodies. We therefore assessed IgG4 immune response in CSS patients.


We included consecutive patients with CSS (n=34, 22 with active and 12 with quiescent disease), granulomatosis with polyangiitis (GPA, n=18) and healthy controls (n=20). We defined the clinical phenotype of CSS patients and determined serum IgG, IgM, IgA and IgE levels. We further assessed IgG subclass distribution and stained tissue biopsies from CSS patients for local IgG4 production.


Whereas active CSS and GPA patients both showed increased total IgG as compared to healthy controls, CSS (mean±SEM: 272±40 mg/dl) but not GPA (mean 90±17 mg/dl) patients showed significantly increased serum IgG4 levels as compared to healthy controls (mean 35±7 mg/dl; p<0.001). Serum IgG4 levels correlated with the number of organ manifestations (p<0.05) and with disease severity, as patients with a Five-Factor Score (FFS)>=1 had higher IgG4 levels than patients with a FFS=0 (p<0.05). IgG4 levels were normal in CSS patients in remission. Heart involvement and peripheral neuropathy were more frequent in IgG4-high (IgG4>135 mg/dL) CSS patients (p=0.13 and p=0.056, respectively). In three out of the nine available CSS tissue biopsies, intense IgG4-producing plasma cell infiltration were detected.


An increased IgG4 production is found in active CSS; IgG4 levels correlate with disease severity, and may differentiate CSS from other vasculitides such as GPA.

To cite this abstract, please use the following information:
Vaglio, Augusto, Strehl, Johanna, Manger, Bernhard, Maritati, Federica, Alberici, Federico, Beyer, Christian, et al; IgG4 Immune Response in Churg-Strauss Syndrome. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :2380

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