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Pan-lobular emphysema due to alpha-1-antitrypsin deficiency

Case contributed by: Dr Jeremy Jones

Presentation:

49 year old male with severe breathlessness and recurrent infections

From the case: Pan-lobular emphysema due to alpha-1-antitrypsin deficiency

Modality: X-ray

Emphysema

Hyper-expansion with flattened diaphragms

From the case: Pan-lobular emphysema due to alpha-1-antitrypsin deficiency

Modality: CT

non-contrast

Pan-lobular emphysema

Widespread severe emphysema with basal predominance.

Case Discussion:

Pan-lobular emphysema with characteristic basal predominance. Severe destruction of the basal segments. Widespread tracheal diverticulae that extend into the lower bronchial tree - associated with severe emphysema.

Subsequent investigation confirmed alpha-1-antitrypsin deficiency.