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[Pulmonary lymphangiomyomatosis].

R Fischbach1, H J Deutsch, K F Neufang

  • 1Institut und Poliklinik für Radiologische Diagnostik, Universität zu Köln.

Aktuelle Radiologie
|January 1, 1991
PubMed
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Pulmonary lymphangiomyomatosis is a rare lung disease. This case study details a 48-year-old woman diagnosed with this condition, highlighting imaging findings and treatment initiation.

Area of Science:

  • Pulmonology
  • Radiology
  • Pathology

Background:

  • Pulmonary lymphangiomyomatosis (PLAM) is a rare, progressive lung disease characterized by the proliferation of smooth muscle cells in the lungs.
  • It primarily affects women of reproductive age and can lead to respiratory compromise.

Observation:

  • A 48-year-old woman presented with symptoms suggestive of interstitial lung disease.
  • Chest radiographs revealed a diffuse reticular infiltrate.
  • High-resolution computed tomography (HRCT) demonstrated multiple, well-defined, diffuse cystic lesions without significant fibrotic changes or nodular densities.

Findings:

  • The patient underwent an open lung biopsy and pleurodesis due to recurrent pneumothorax.
  • Histopathological examination confirmed pulmonary lymphangiomyomatosis.

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  • Treatment with medroxyprogesterone was initiated for the condition.

    • Implications:

    • This case highlights the importance of HRCT in diagnosing PLAM by visualizing characteristic cystic changes.
    • Early diagnosis and management, including hormonal therapy, may be crucial for patients with PLAM.
    • Further research into optimal treatment strategies for pulmonary lymphangiomyomatosis is warranted.