A report of two long-surviving cases of pulmonary lymphangioleiomyomatosis and the response to progesterone therapy

Br J Dis Chest. 1985 Oct;79(4):400-6. doi: 10.1016/0007-0971(85)90077-4.

Abstract

Two cases of pulmonary lymphangioleiomyomatosis, with survival between 12 and 28 years are described. Both were treated with medroxyprogesterone acetate with a slight initial improvement in both lung function and exercise tolerance, and no further deterioration during the period of follow-up of 10-20 months. The results are discussed and other reports of hormonal treatment of this rare condition are reviewed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Follow-Up Studies
  • Humans
  • Lung / physiopathology
  • Lung Neoplasms / drug therapy*
  • Lung Neoplasms / physiopathology
  • Lymphangiomyoma / drug therapy*
  • Lymphangiomyoma / physiopathology
  • Lymphoproliferative Disorders / drug therapy*
  • Medroxyprogesterone / analogs & derivatives*
  • Medroxyprogesterone / therapeutic use
  • Medroxyprogesterone Acetate
  • Respiratory Function Tests

Substances

  • Medroxyprogesterone Acetate
  • Medroxyprogesterone