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IgG4 immune response in Churg–Strauss syndrome
  1. Augusto Vaglio1,
  2. Johanna D Strehl2,
  3. Bernhard Manger3,
  4. Federica Maritati1,
  5. Federico Alberici1,
  6. Christian Beyer3,
  7. Jürgen Rech3,
  8. Renato A Sinico4,
  9. Francesco Bonatti1,
  10. Luisita Battistelli5,
  11. Jörg H W Distler3,
  12. Georg Schett3,
  13. Jochen Zwerina3,6
  1. 1Department of Clinical Medicine, Nephrology and Health Sciences, University of Parma, Parma, Italy
  2. 2Department of Pathology, University of Erlangen-Nuremberg, Erlangen, Germany
  3. 3Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany
  4. 4Nephrology and Clinical Immunology Unit, San Carlo Borromeo Hospital, Milan, Italy
  5. 5Department of Laboratory Medicine, University Hospital of Parma, Parma, Italy
  6. 6Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria
  1. Correspondence to Jochen Zwerina, Department of Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Krankenhausstrasse 12, D-91054 Erlangen, Germany; jochen.zwerina{at}


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.

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  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the University of Erlangen and the University of Parma.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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