The relationship between atopy and cystic fibrosis.
The incidence and clinical significance of allergy in cystic fibrosis have been discussed. There is little evidence that the high prevalence of positive allergy skin tests in CF is a clinical manifestation of a hypersensitivity lung disease complicating the primary pulmonary disorder, except in the special case of allergic bronchopulmonary aspergillosis. The lung disease of CF appears to be caused by excessive bronchial secretions and recurrent infection that are the result of abnormal ion transport across the apical membrane of the respiratory epithelial cell. There are two important questions concerning the allergic reactions: Are they clinically significant, and, why do they occur? The former question is partly resolved by the data presented, although well-controlled studies of intervention would help to resolve it further. The cause of these reactions, which occur particularly in relationship to the mold Aspergillus fumigatus, is unknown but the author is inclined to the view that they are the result of recurrent infection that induces heightened immunologic reactivity to inhaled allergens. The significance of AF may be simply that this mold has a "predilection for diseased airways" or may be more complex; for example, owing to abnormal ion composition in respiratory secretions, abnormal lectin expression by CF respiratory epithelial cells, or selective filtration by the airways based on particle size.
Wilmott
R W
RW
Children's Hospital Medical Center, Cincinnati, OH 45229.
D003550:Cystic Fibrosis / Q000276:immunology*; D006801:Humans; D006969:Hypersensitivity, Immediate / Q000453:epidemiology*; D015995:Prevalence