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Pulmonary lymphangioleiomyomatosis

K Viskum1

  • 1Dept of Pulmonary Medicine, Bispebjerg Hospital, Copenhagen, Denmark.

Monaldi Archives for Chest Disease = Archivio Monaldi Per Le Malattie Del Torace
|January 1, 1993
PubMed
Summary

Lymphangioleiomyomatosis (LAM) is a rare lung disease primarily affecting women. Treatment involves hormonal therapies like medroxyprogesterone or oophorectomy, with a cautiously improving prognosis.

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Area of Science:

  • Pulmonology
  • Oncology
  • Endocrinology

Background:

  • Lymphangioleiomyomatosis (LAM) is a rare, progressive interstitial lung disease.
  • It predominantly affects women of childbearing age and is associated with hormonal influences.
  • The exact pathogenic mechanisms and hormonal dependencies remain incompletely understood.

Purpose of the Study:

  • To review the diagnosis, symptoms, and diagnostic investigations for LAM.
  • To explore the role of hormonal dependency and receptor status in LAM.
  • To evaluate the efficacy of current hormonal treatments and surgical interventions.

Main Methods:

  • Review of diagnostic criteria and clinical presentation of LAM.
  • Analysis of paraclinical investigations including CT scans and spirometry.
  • Assessment of treatment outcomes with medroxyprogesterone and bilateral oophorectomy.

Main Results:

  • Hormonal dependency is evident, though mechanisms are unclear; estrogen and progesterone receptor status in myoma tissue is variable.
  • Receptor status does not appear to influence treatment outcomes.
  • Medroxyprogesterone treatment and oophorectomy show uncertain but potentially beneficial results.

Conclusions:

  • A therapeutic trial with medroxyprogesterone is warranted for LAM patients.
  • Bilateral oophorectomy is recommended if medroxyprogesterone proves ineffective.
  • While respiratory insufficiency remains a risk, the prognosis for LAM appears to be improving, with long-term survivors documented.

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