Abstract | PURPOSE OF REVIEW: RECENT FINDINGS: In two studies, angiomyolipoma size decreased by 26-50% after 12 months of sirolimus treatment. In a recent 12 months controlled trial involving 89 patients with pulmonary LAM, sirolimus stopped lung function decline and improved quality of life and performance score. The protective effect of sirolimus was lost after treatment discontinuation, with a parallel lung function decline in both groups, similar to the increase in angiomyolipoma size. Sirolimus is associated with an excess of adverse events. SUMMARY:
Sirolimus represents an important drug for LAM that should be proposed to patients with a rapid alteration of lung function or with a significant clinical impairment, after individual evaluation of the risk/benefit ratio. Sirolimus seems to have a sharper effect on the reduction of abdominal masses than on lung cysts. Tolerance and safety concerns are serious limits to the long-term treatment of patients with sirolimus.
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Authors | Camille Taillé, Raphaël Borie, Bruno Crestani |
Journal | Current opinion in pulmonary medicine
(Curr Opin Pulm Med)
Vol. 17
Issue 5
Pg. 374-8
(Sep 2011)
ISSN: 1531-6971 [Electronic] United States |
PMID | 21760507
(Publication Type: Journal Article, Review)
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Chemical References |
- Anti-Bacterial Agents
- Immunosuppressive Agents
- Doxycycline
- Sirolimus
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Topics |
- Anti-Bacterial Agents
(therapeutic use)
- Doxycycline
(therapeutic use)
- Humans
- Immunosuppressive Agents
(adverse effects, therapeutic use)
- Lymphangioleiomyomatosis
(drug therapy, pathology)
- Quality of Life
- Sirolimus
(adverse effects, therapeutic use)
- Treatment Outcome
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