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Pulmonary Surfactant, Lung Function, and Endobronchial Inflammation in Cystic Fibrosis

Children's Hospital, University of Munich, Munich; Internal Medicine, University of Giessen, Giessen; Department of Pediatric Pneumology and Allergology, Children's Hospital, University of Cologne, Cologne; Children's Hospital, University of Essen, Essen; Department of Pediatric Pneumology, Medical School, University of Hannover, Hannover; and Department of Pediatric Pneumology and Immunology, Charité, Humboldt-University, Berlin, Germany

Correspondence and requests for reprints should be addressed to Professor Matthias Griese, M.D., Children's Hospital, University of Munich, Lindwurmstr 4, 80337 Munich, Germany. E-mail: matthias.griese{at}med.uni-muenchen.de

Cystic fibrosis (CF) lung disease is primarily a disease of the small airways. We hypothesized that even in patients with normal lung function, a reduced surfactant function would be present and favor small airway obstruction. Bronchoalveolar lavages from 76 patients with CF (5–31 years, median 11) with well-conserved lung function (FEV₁ 94% predicted, range 78–121) and from 10 healthy control subjects were investigated. The deviation of the biophysical surfactant performance from normal, assessed in a bubble surfactometer, was small; however, the ability of the surfactant to maintain the patency of a narrow airway (% open) was significantly reduced. Surfactant protein (SP)-C level was increased, SP-B and SP-D were unchanged, whereas SP-A was decreased. Among the patients with CF, neutrophilic inflammation was modestly related to a poorer surfactant activity, but not to lung function. SP-D was reduced in proportion to the degree of inflammation and in the presence of bacteria. These findings in a large cohort of patients with CF with normal lung function show that the endobronchial airway inflammation is linked to early perturbations of the biophysical properties and immunologic components of pulmonary surfactant and opens fields for novel therapeutic interventions.

Key Words: airway inflammation • bronchoalveolar lavage • capillary surfactometer • pulsating bubble • surface activity

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