Question:medium
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 conditions was most likely associated with the pathologic changes shown here?
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 conditions was most likely associated with the pathologic changes shown here?
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Emphysema in a young non-smoker points to a missing protease inhibitor.
Updated On: Jun 23, 2026
- Mutation in dynein arms
- Cystic fibrosis
- Alpha-1 antitrypsin deficiency
- Antibodies against type 4 collagen
Show Solution
The Correct Option is C
Solution and Explanation
The trap-buster here is the phrase not a smoker. Emphysema in a young person who has never smoked is alpha-1 antitrypsin deficiency until proven otherwise, because cigarette smoke is the usual driver and its absence forces a genetic explanation.
Mechanism in one line: alpha-1 antitrypsin is the main inhibitor of neutrophil elastase. Lose it, and elastase digests alveolar walls unchecked, giving panacinar emphysema (often lower-zone predominant) plus, in some patients, liver disease from accumulation of misfolded protein in hepatocytes.
Sweep the options: dynein-arm mutation gives Kartagener primary ciliary dyskinesia with bronchiectasis and situs inversus, cystic fibrosis gives sticky-secretion bronchiectasis, and anti-type-IV-collagen antibodies give Goodpasture pulmonary-renal syndrome, none of which produce isolated panacinar emphysema in a non-smoker. The answer is option (c), alpha-1 antitrypsin deficiency.
Mechanism in one line: alpha-1 antitrypsin is the main inhibitor of neutrophil elastase. Lose it, and elastase digests alveolar walls unchecked, giving panacinar emphysema (often lower-zone predominant) plus, in some patients, liver disease from accumulation of misfolded protein in hepatocytes.
Sweep the options: dynein-arm mutation gives Kartagener primary ciliary dyskinesia with bronchiectasis and situs inversus, cystic fibrosis gives sticky-secretion bronchiectasis, and anti-type-IV-collagen antibodies give Goodpasture pulmonary-renal syndrome, none of which produce isolated panacinar emphysema in a non-smoker. The answer is option (c), alpha-1 antitrypsin deficiency.
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