LAM can have normal PFT until late in disease

The majority of patients with LAM have normal physiologic testing, with the following in order of frequency: reduction in DLCO, obstructive pattern, and restrictive or mixed obstructive and restrictive pattern. Progressive decline in lung function occurs as dysregulated smooth musclelike cells (LAM cells) infiltrate the lung, disrupting lymphatics and leading to parenchymal destruction and cyst formation.1

Footnotes

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