Pulmonary cystic fibrosis in the adult: early and late radiologic findings with pathologic correlations

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Abstract
Radiographs of 50 patients age 17 or more with documented cystic fibrosis were reviewed. Peripheral nodular and nonvascular linear densities were common early abnormalities. Specific findings of bronchiectasis were found in 90% of all cases. Hyperinflation was seen in 76% of cases, especially in the lower lobes; atelectasis and all other abnormalities were more common in the upper (and middle) lobes. Cystic air spaces developed in 24% of cases. The severity of abnormalities (including hyperinflation, atelectasis, and bronchial changes) increased in 30 of the 39 patients with follow up for a year or more. Eight of the 15 patients who died came to autopsy. The lungs showed acute and chronic inflammation of airways, including peripheral bronchioles, with adjacent parenchymal inflammation of airways peripheral bronchioles, with adjacent parenchymal infiltrates and fibrosis. The surrounding alveoli were aerated and enhanced the visibility of the thick-walled airways, except in regions of lobar atelectasis, scarring, or active pneumonia. Large and small airway shadows can be described more precisely than by the terms "honeycombing," "interstitial" or "bronchovascular markings".