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Sirolimus Therapy in Patients With Lymphangioleiomyomatosis FREE

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The full report is titled “Changes in Lung Function and Chylous Effusions in Patients With Lymphangioleiomyomatosis Treated With Sirolimus.” It is in the 21 June 2011 issue of Annals of Internal Medicine (volume 154, pages 797-805). The authors are A.M. Taveira-DaSilva, O. Hathaway, M. Stylianou, and J. Moss.

Ann Intern Med. 2011;154(12):I-44. doi:10.7326/0003-4819-154-12-201106210-00003
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What is the problem and what is known about it so far?

Lymphangioleiomyomatosis (LAM) is a disease caused by the abnormal growth of specific muscle cells known as smooth muscle cells within certain organs. This disease can cause progressive shortness of breath by destroying lung tissue and, in some patients, by causing accumulation of fluid around the lung known as pleural effusion. These effusions occur when normal removal of this fluid is blocked. The disease can also block the drainage of fluid in the abdomen, which causes ascites, and can cause abnormal growths that impair kidney function.

Why did the researchers do this particular study?

To determine whether the drug sirolimus, which can control the growth of smooth muscle cells, can help to improve lung function or decrease the size of pleural effusions in patients with LAM.

Who was studied?

19 patients with progressively debilitating LAM who chose to try sirolimus therapy while participating in a National Institutes of Health study that was designed to observe the natural course of LAM.

How was the study done?

The patients underwent repeated lung function studies and x-rays as part of the National Institutes of Health study. Researchers compared the changes in lung function and pleural effusions that occurred before and after starting sirolimus therapy.

What did the researchers find?

During an average of 2.5 years before patients started sirolimus therapy, lung function progressively worsened. In contrast, lung function improved overall during an average of 2.6 years of sirolimus treatment. Many patients with pleural effusions required repeated drainage of the fluid with a needle before treatment and subsequently experienced an improvement with less fluid accumulation or resolution of the fluid altogether while receiving sirolimus therapy. Patients without effusions experienced stabilization of their lung function during sirolimus treatment.

What were the limitations of the study?

This was an observational study, and whether sirolimus was the cause of the change in lung function and pleural effusions cannot be definitively established.

What are the implications of the study?

Sirolimus might be considered for the treatment of patients with LAM who have progressively worsening lung function or recurrent pleural effusions.





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