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Severe impaired respiratory ciliary function in Wegener granulomatosis
  1. S Ullrich1,
  2. H Gustke1,
  3. P Lamprecht2,
  4. W L Gross2,
  5. U Schumacher1,
  6. P Ambrosch3,
  7. M Laudien3
  1. 1
    Department for Anatomy II, Experimental Morphologie, University Medical Center Hamburg–Eppendorf, Hamburg, Germany
  2. 2
    University of Lübeck, Department for Rheumatology, Vasculitis Center UKSH & Rheumaklinik Bad Bramstedt, Lübeck, Germany
  3. 3
    Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Kiel, Germany
  1. S Ullrich, Department for Anatomy II: Experimental Morphology, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, D-20246 Hamburg, Germany; hsullrich{at}


Objective: The pathogenesis of granulomatous inflammation in the respiratory tract and autoimmunity in Wegener granulomatosis (WG) are poorly understood. Since mucociliar clearance represents the first major line of defence in the respiratory tract and its breakdown facilitates chronic inflammation, we investigated ciliary beat frequency (CBF) in WG.

Methods: Nasal epithelial cells were obtained from 30 patients with WG with involvement of the upper respiratory tract, 12 patients with other inflammatory rheumatic disease and 10 healthy controls. CBF was measured at 5 and 24 h after collection. Results were correlated with clinical data.

Results: CBF was significantly reduced in WG compared to disease and healthy controls after 5 and 24 h. In WG, CBF almost stagnated after 24 h. Reduction of CBF correlated with the cumulative number of immunosuppressive agents in WG, but not in disease controls. No correlation was found between CBF impairment and cyclophosphamide levels, disease extent, disease activity, disease duration, serological and microbiological findings, or inflammation markers.

Conclusion: CBF is severely impaired in WG, potentially influenced by immunosuppressive treatment. To what extent CBF impairment and subsequent barrier dysfunction are caused by other factors still has to be elucidated. Supportive measures to improve mucociliary clearance should be discussed in patients with WG.

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  • Competing interests: None declared.

  • Funding: This work was supported by German Research Foundation (DFG)-funded Clinical Research Unit/KFO170 and Foundation for the Promotion of Research and Treatment of Rheumatic Diseases, Bad Bramstedt.

  • Ethics approval: The study protocol was approved by the ethic committees of the universities of Lübeck, Kiel and Hamburg.

  • ▸ Additional data (supplementary video 1 and supplementary fig 1) are published online only at

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