Alpha-1-antitrypsin deficiency

Case contributed by Dr Andrew Dixon

Presentation

Worsening shortness of breath

Patient Data

Age: 35 years
Gender: Female
X-ray

Marked hyperinflation of the lungs (note over 11 posterior ribs visible on PA projection, flattened hemidiaphragms, increased retrosternal air space) and hyperlucency of the lungs with distorted parenchymal architecture.

Annotated images

Annotated image

Both lungs are markedly hyperinflated with 11 posterior ribs easily visible above the hemidiaphragmatic domes (yellow dotted lines). The hemidiaphragms are depressed and flattened (best seen on lateral projection, with loss of the normal acute angle formed by the costophrenic pleural reflection (dotted blue line).

The lungs are very radiolucent with some areas devoid of lung markings (*) suggesting bullous formation.

On lateral projection the retrosternal air space (RS) is markedly widened (aorta outlined in red).

Diagram

Diagram of the three types of emphysema. 

Case Discussion

Severe mid and lower zone emphysema (panlobular) in a patient with alpha-1-antitrypsin deficiency. Note the basal predominance which helps differentiate this condition from the far more common centrilobular emphysema of smoking.

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