Lymphangioleiomyomatosis

Case contributed by Dr Mostafa Mohamed

Presentation

Chronic dyspnea for 20 years.

Patient Data

Age: 50 years
Gender: Female
CT

Bilateral, diffuse, variable-sized lung cysts with apparent ground glass opacification of the intervening lung representing walls of micro-cystic lesions.

Rather prominent major fissures (slightly fibrotic).

Complementary mediastinal images show prominent main pulmonary artery (about 4 cm).

Case Discussion

Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease caused by bronchiolar obstruction and lung destruction due to proliferation of immature smooth muscle cells in small vessels, lymphatics and bronchioles.

Almost all cases occur in females in childbearing age. About 1% of patients with tuberous sclerosis have lung disease similar to LAM.

Differential diagnosis includes:

  • Langerhans cell histiocytosis (LCH): cysts tend to occur in upper lobes
  • lymphocytic interstitial pneumonia: cysts associated with fibrosis
  • cystic bronchiectasis:characterized by saccular dilatation of bronhi extending to pleura
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Case information

rID: 71904
Published: 5th Nov 2019
Last edited: 5th Nov 2019
System: Chest
Inclusion in quiz mode: Included

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