title: Efficacy of hormonal manipulation in lymphangioleiomyomatosis. A 20-year-experience in 36 patients.
abstract: BACKGROUND
  Lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology which mainly affects women of reproductive age. A growing body of evidence suggests a relation between the disease and estrogenic hormones. In particular, pregnancy and use of estrogens favour the progression of the disease, which is in turn slowed down by menopause. These observations suggested already in the past a treatment strategy based on hormonal manipulation: discontinuation of estrogens containing medications, avoiding pregnancy, inhibition of estrogens activity and induction of menopause.
  
  
  OBJECTIVE
  In the present study, conducted on a relatively large population for a rare disease, we sought to show the hormonal manipulation's positive effects for the survival rate.
  
  
  METHODS
  36 women suffering from LAM were evaluated between January 1985 and March 2005. All our patients were treated with hormonal therapy, following different schemes. The response to the treatment was evaluated in all patients with arterial blood sampling, lung function tests (total body plethysmography, DLCO), measurement of the hormones in the blood, chest and abdomen CT scan.
  
  
  RESULTS
  The survival rate since the clinical onset was 97% at the 5 year timepoint, 90% at 10 years, and 71% at 25 years. Previous studies, conducted before the introduction of the hormonal treatment showed a survival rate of 20% at 10 years.
  
  
  CONCLUSIONS
  Our results highlight the role of the hormonal manipulation as a mainstay in the treatment of LAM, with the capability of reducing mortality and improving the quality of life as well.
authors: Respiratory Pathophysiology Unit, S. Orsola-Malpighi Hospital, Via Massarenti 9 40138 Bologna, Italy. mschiavina@aosp.bo.it
authors: Mario
authors: M
authors: Schiavina
authors: Paola
authors: P
authors: Contini
authors: Andrea
authors: A
authors: Fabiani
authors: Franca
authors: F
authors: Cinelli
authors: Valerio
authors: V
authors: Di Scioscio
authors: Maurizio
authors: M
authors: Zompatori
authors: Cristina
authors: C
authors: Campidelli
authors: Stefano A
authors: SA
authors: Pileri
mesh_terms: D000328:Adult; D004965:Estrogen Antagonists / Q000008:administration & dosage / Q000627:therapeutic use*; D004967:Estrogens / Q000378:metabolism*; D005260:Female; D006728:Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D016729:Leuprolide / Q000008:administration & dosage / Q000627:therapeutic use; D008186:Luteolytic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D018192:Lymphangioleiomyomatosis / Q000188:drug therapy / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy*; D017258:Medroxyprogesterone Acetate / Q000008:administration & dosage / Q000627:therapeutic use; D008593:Menopause; D008875:Middle Aged; D010052:Ovariectomy; D012129:Respiratory Function Tests; D017329:Triptorelin Pamoate / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D004965:Estrogen Antagonists; D004967:Estrogens; D006728:Hormones; D008186:Luteolytic Agents; D017329:Triptorelin Pamoate; D017258:Medroxyprogesterone Acetate; D016729:Leuprolide