Lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis),
LAM, is a rare lung disease that affects women almost exclusively. LAM is characterized by an abnormal growth of smooth muscle cells, especially in the lungs, lymphatic system, and kidneys. Abnormal growth of these cells can lead to loss of lung function, accumulation of lymph rich fluid in the chest and abdomen, and growth of benign tumors in the kidneys. Sirolimus (mTOR inhibitor) is the first-line treatment option for managing symptoms and complications of LAM.
- Patients with LAM should maintain appropriate vaccinations.
- Live vaccines should be avoided in patients taking immunosuppressive agents, so vaccination recommendations differ for LAM patients who are on mTOR inhibitors such as sirolimus and those who are not.
- Based on available evidence, it is not recommended that sirolimus be held prior to receiving the COVID vaccine.
See updates for COVID-19 and individuals with LAM
HPS Research Studies
This is a short video clip about why people with HPS volunteer to participate in medical research studies.
HPS Research
This links to a list of current and former studies involving patients with Hermansky-Pudlak Syndrome.
ATS Patient Information Series
The ATS Patient Information Series is a public service of the American Thoracic Society and its journal the AJRCCM (www.atsjournals.org). The information appearing in this series is for educational purposes only and should not be used as a substitute for the medical advice of one's personal health care provider.
