Combined pulmonary fibrosis and emphysema

Case contributed by Domenico Nicoletti


Exertional dyspnea, cough, perioral cyanosis, and asthenia.

Patient Data

Age: 75 years
Gender: Male

Emphysema in the upper fields and reticular shadow in the lower fields.

Presence of paraseptal emphysema, centrilobular emphysema and subpleural bullae in both upper lobes with reticular opacities, bronchiolectasis and typical honeycombing. In the right lower lobe, in the azygoesophageal recess, there is also a parenchymal nodule. There is mediastinal adenopathy.

Bronchoalveolar lavage

Clinical history: pulmonary emphysema in smoker with diffuse infiltrative pneumopathies of smooth character with areas of increased density especially at the bases. CPFE ? DIP? RBILD? Microscopic description macrophages, bronchial cells and some rare neutrophil granulocytes. Diagnosis: see microscopic description.

Diffusion lung CO


Diffusion capacity for carbon monoxide (DLCO) is significantly reduced (32%)

Case Discussion

The syndrome resulting from combined pulmonary fibrosis and emphysema (CPFE) is related to cigarette smoking, although the pathophysiology remain to be determined. The upper lobe emphysematous lesions in CPFE include centrilobular emphysema, paraseptal emphysema and bullae; frequently it is complicated by pulmonary hypertension. In CPFE DLco is severely diminished.

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