Imaging diagnosis of interstitial pneumonia with emphysema (combined pulmonary fibrosis and emphysema)

Abstract

Based on clinical and radiological findings, Cottin defined combined pulmonary fibrosis and emphysema (CPFE) as pulmonary emphysema in the upper lungs and interstitial pneumonia in the lower lungs with various radiological patterns. Pathologic findings of CPFE probably corresponded with diffuse interstitial pneumonia with pulmonary emphysema, emphysema with fibrosis, and the combination of both. We described reported radiological findings of CPFE.

Figure 1

Diffuse interstitial pneumonia (nonspecific interstitial pneumonia (NSIP) pattern) with emphysema. (a) Chest X-ray shows bibasilar ground glass and reticular opacity. Volumes of bilateral lungs are almost normal. (b) High-resolution computed tomography (HRCT) of upper lung shows centrilobular pulmonary emphysema. (c), (d) HRCT of lower lung shows ground glass opacity along the bronchovascular bundle including cysts with varying size. Distribution of abnormal opacity mimics NSIP.

Figure 2

Diffuse interstitial pneumonia (UIP pattern) with emphysema. (a) Chest X-ray shows decreased volume of bilateral lungs. Ground glass opacity including reticular opacity and honeycomb lung in bilateral lower lungs. (b) HRCT of the right upper lobe shows paraseptal emphysema in ventral aspect. (c) HRCT of the right lower lobe shows ground glass opacity and honeycomb lung. Traction bronchiectasis is also observed within the areas of honeycombing.

Figure 3

Diffuse interstitial pneumonia (UIP pattern), emphysema with fibrosis. The patient underwent right lower lobectomy for lung cancer of the right lower lobe. (a) HRCT of the right upper lobe shows severe centrilobular emphysema. (b) HRCT of the right lower lobe shows slight ground glass opacity including several thick-walled cyst. (c) HRCT of basilar region shows subpleural ground glass opacity and reticular opacity in the subpleural region: radiologically consistent with UIP pattern. There are some relatively thick-walled cysts in subpleural region; however, no evidence of honeycombing. (d) Pathologic specimen (H&E staining) shows centrilobular and paraseptal emphysema with collagen type fibrosis. (e) Pathologic specimen shows dense centrilobular collagen-type fibrosis, associated with centrilobular emphysema. (f) Pathologic specimen shows cystic lesion in the acinus with destruction of alveoli and dense perilobular and peribronchiolar fibrosis. Normal alveoli adjacent to the cystic lesion. (g) Pathologic specimen shows patchy subpleural and intralobular fibrosis with alternating emphysematous, nonfibrotic area. Note fibroblastic foci (arrows). Sharp border between advanced fibrosis and normally appeared tissue suggests UIP pattern.

Figure 4

Emphysema with fibrosis, thick-walled large cyst. (a) HRCT of upper lungs shows severe centrilobular emphysema. A relatively thick-walled cyst in the superior basal segment of the left lower lobe. Lung cancer is noted in the lingual division of the left upper lobe. (b) HRCT of left lower lung shows large thick-walled cysts surrounded by ground glass opacity in the regions apart form pleural surface. (c) HRCT of left lower lobe shows multiple bizarre-shaped cysts aggregated in the central portion of the left lower lobe. (d) Pathologic specimens of the resected left upper lobe shows centrilobular cystic lesion involving the membranous and respiratory bronchioles with fibrosis. Note peripheral lung parenchyma spared. (e) Mucus filling in the alveoli with slight septal thickening adjacent to the cyst (circle of Figure 5(e)). Mucus filling and septal thickening correspond to ground glass opacity surrounding large cysts.

Figure 5

Airway-centered fibrosis with cysts. (a) Chest X-ray shows increased volume of bilateral lungs. (b) High-resolution computed tomography (HRCT) of lower lung shows ground glass opacity and multiple cysts in subpleural region, but these abnormal opacities are predominant in centrilobular region (centrilobular accentuation).

Figure 6

Emphysema with fibrosis, pulmonary hypertension. Estimated right ventricular pressure by cardiac ultrasound is 80 mmHg. (a) Chest X-ray shows cardiomegaly with enlargement of central pulmonary arteries. (b) Contrast-enhanced CT shows dilatation of central pulmonary arteries. There is no evidence of pulmonary thromboembolism. (c) High-resolution computed tomography (HRCT) of upper lung shows severe pulmonary emphysema; some cystic lesions have thick wall. (d) HRCT of the lower lung shows marked destructive change in the lung. There are several large cysts with thick wall.

Figure 7

Emphysema with fibrosis, acute exacerbation by anticancer drug. (a)–(e) Imaging findings before acute exacerbation. (a) Chest X-ray shows normal volume of bilateral lungs. There is reticular opacity in bilateral lower lung fields. (b), (c) High-resolution CT (HRCT). In upper lung, centrilobular emphysema, subpleural cyst, and profuse centrilobular small nodular opacities are identified. In lower lung, subpleural cysts and reticular opacity are noted abutting chest wall, most compatible with UIP pattern. (d)–(f) Imaging findings at acute exacerbation. (d) Chest X-ray shows diffuse ground glass opacity in the left entire lung. Pneumothorax is noted on right side. (e), (f) HRCT shows diffuse ground glass opacity overlapping preexisting interstitial shadow.