title: Severe impaired respiratory ciliary function in Wegener granulomatosis.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department for Anatomy II, Experimental Morphologie, University Medical Center Hamburg-Eppendorf, Germany. hsullrich@aol.com
authors.forename: S
authors.initials: S
authors.lastname: Ullrich
authors.forename: H
authors.initials: H
authors.lastname: Gustke
authors.forename: P
authors.initials: P
authors.lastname: Lamprecht
authors.forename: W L
authors.initials: WL
authors.lastname: Gross
authors.forename: U
authors.initials: U
authors.lastname: Schumacher
authors.forename: P
authors.initials: P
authors.lastname: Ambrosch
authors.forename: M
authors.initials: M
authors.lastname: Laudien
mesh_terms: D000704:Analysis of Variance; D015551:Autoimmunity / Q000502:physiology; D016022:Case-Control Studies; D002923:Cilia / Q000502:physiology* / Q000648:ultrastructure; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009079:Mucociliary Clearance; D009297:Nasal Mucosa / Q000473:pathology / Q000648:ultrastructure*; D013997:Time Factors
chemical_list: D007166:Immunosuppressive Agents