The progression of lung disease in patients with cystic fibrosis (CF) was evaluated with chest radiography. The severity and extent of the various radiographic changes were scored with the Chrispin or the Birmingham method, which involves the use of imprecise and subjective terms, such as line shadows, large pulmonary shadows, and nodular cystic lesions. Although computed tomography (CT) has been shown to be helpful in the evaluation of lung disease in CF, no scoring system or other objective criteria have been developed for the evaluation of the wide range of pulmonary changes in these patients. A CT scoring system was devised that incorporates all of the changes seen in the lungs of patients with CF. Such a scoring system may facilitate objective evaluation of existing and newly developed therapeutic regimens and may be a valuable tool in the preoperative evaluation of patients being considered for lobectomy or bullectomy and in the selection of patients for lung transplantation.

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Although better insights into the natural course of cystic fibrosis (CF) have led to treatment approaches that have improved pulmonary health and increased the life expectancy of individuals with this disorder, lung disease remains the main cause of morbidity and mortality in patients with CF. Evidence suggests that airway epithelial defects in ions-water transport lead to dehydrated mucus, impaired mucus clearance, and mucus adhesion to airway surfaces. An increase in mucin secretion is also suggested by the formation of endobronchial mucus plaques and plugs, which become the main sites of air flow obstruction, infection, and inflammation conducing to early small airways disease followed by the development of bronchiectasis. The lung involvement is usually progressive with intermittent exacerbations. Aggressive management and advances in treatment delay, but, do not prevent progression of lung disease. Respiratory failure ensues and is the major cause of death. The lung parenchyma is virtually untouched for much of the course of the disease. This review focuses on the lung involvement in cystic fibrosis and summarizes new developments on the diagnostic approach of CF and pathogenesis of related lung disease. Current therapeutic modalities, novel therapies targeting the basic genetic defect, and lung transplantation are also reviewed.

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