Lymphangioleiomyomatosis - LAM
Clinical/Pathology: A disease of young women, often of African origin characterized by proliferation of lymphatic and myoepithelial tissue around small airways. Structural lung damage occurs with focal air-trapping, hyperinflation, small regional cystic changes and impairment of lymph flow.
CXR/CT Findings: The classic pattern is quite diagnostic.
- small diffuse, bilateral thin walled lung cysts with fine interstitial changes
- lung hyperinflation
- sometimes pleural effusions
- significant adenopathy and lung nodules are not seen
Clues: A bilateral uniform thin walled cystic pattern in young women. A high resolution CT without contrast should substantiate diagnosis.
Treatment: Diagnosis is vital for patient prognosis and management as the only definitive therapy is lung transplant.
"Aunt Sophies": Usually considered only in mild or somewhat atypical presentations.
- Eosinophilic granuloma: most often in male smokers. Upper lung cysts with nodules and no pleural reaction.
- Interstitial Fibrosis: end stage interstitial lung disease with diffuse honeycombing may superficially mimic LAM. Older patients, fibrosis, volume loss, not uniform
- Diffuse bronchiectasis: often in immunologically or structurally compromised hosts (eg. cilia dyskinesia syndrome)
- Cystic fibrosis
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