A 36-yr-old woman with systemic sclerosis develops breathlessness on exertion. Her pulmonary function tests show normal spirometry but a decreased gas transfer factor (TLCO, transfer factor for carbon monoxide) and transfer coefficient (KCO).
Which of the following is the most likely explanation for this abnormality?
A. Fibrosing alveolitis
B. Pulmonary vascular disease
C. Severe thoracic skin thickening
D. Pleural involvement
E. Respiratory muscle weakness
Answer:
B) Pulmonary vascular disease
Discussion: Isolated decreases in gas transfer are typical of pulmonary vascular disease, eg vasculitis,
recurrent pulmonary embolism (PE).
In fibrosing alveolitis you would also expect to see decreased lung volumes with a restrictive ratio (> 80%) on spirometry.
The other three answers would all give a picture of ‘extrapulmonary restriction’ with a restrictive
ratio, low TLCO but normal/high KCO (same cardiac output going through smaller alveolar volume).
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