Lymphangioleimyomatosis: Clinical course

M. Schiavina, A. Fabiani, B. Cornia, E. DeBenedictis, V. Poletti, S. Pileri, M. Zompatori

Research output: Contribution to journalArticlepeer-review

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of smooth muscle proliferation that generally leads to death, due to respiratory failure, within 10 yrs. The disease almost exclusively affects women of child-bearing age and rarely in postmenopausal years. High resolution computed tomographic (CT) patterns are characteristic and almost pathognomonic for LAM. Attempts to treat this disease with hormonal therapy have shown that a number of hormonal manipulations may not be helpful, but that surgical or radiant oophorectomy alone is the most effective treatment, even if the disease is so rare as to preclude randomized trials. Castration, as a means of early therapy, has been associated with a stable respiratory picture in our patients.

Original languageEnglish
Pages (from-to)6-14
Number of pages9
JournalMonaldi Archives for Chest Disease - Cardiac Series
Volume49
Issue number1
Publication statusPublished - 1994

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pulmonary and Respiratory Medicine

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