Question
A 35-year-old woman with a long history of dyspnea, chronic cough, sputum production, and wheezing dies of respiratory failure following a bout of lobar pneumonia. She was not a smoker or an alcoholic. The lung autopsy is shown in the image. Which of the following underlying condition was most likely associated with the pathologic changes shown here?
| A. |
Mutation in dynein arms
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| B. |
Cystic fibrosis
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| C. |
Alpha 1 antitrypsin deficiency
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| D. |
Antibodies against type 4 collagen
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Show Answer
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Correct Answer � C
Explanation
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Ans: C. Alpha 1 antitrypsin deficiency
The photomicrograph above is suggestive of hereditary alpha-1-antitrypsin deficiency. Alpha-1-antitrypsin is normally present in serum, tissue fluids, and macrophages. It’s an inhibitor of proteases (which are destructive enzymes secreted by neutrophils during inflammation).
Patients who are deficient in alpha-1-antitrypsin are less able to counter the effects of proteases, and the elastic tissue in the lung is eventually destroyed, producing emphysema (irreversible enlargement of the airspaces distal to the terminal bronchiole).
In addition to emphysema, patients with an alpha-1-antitrypsin deficiency may develop hepatitis and even cirrhosis from the accumulation of abnormally-folded alpha-1-antitrypsin within hepatocytes.
These proteins are visible as brightly eosinophilic inclusions with regular hematoxylin and eosin staining. The disease varies in its presentation; some cases are severe at birth, and others are asymptomatic until later life. In patients with severe liver disease, liver transplantation is the treatment of choice.
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