Objective: The pathogenesis of granulomatous inflammation in the respiratory tract and autoimmunity in Wegener's 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 the ciliary beat frequency (CBF) in WG.
Methods: Nasal epithelial cells were obtained from 30 WG-patients 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 hours 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 hours. In WG, CBF almost stagnated after 24 hours. 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, disease extent, -activity, -duration, serologic and microbiologic 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 WG-patients.
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Review history and Supplementary material
Web only appendix 68;6:1067-1071
Web only appendix 68:6;1067-1071
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