LAM

LAM Week

LAM Week

Welcome Message

Pulmonary lymphangioleiomyomatosis (LAM) is a progressive neoplastic genetic disease affecting predominantly women.  The disease is characterized by the infiltration of atypical smooth muscle LAM cells in the lung and lymphatics, and cystic remodeling of lung parenchyma.  Obstruction of lymphatics might result in the collection of chylous fluid and fluid-filled lymphangiomyomas in the chest and abdomen. The angiomyolipoma, a benign tumor composed of smooth muscle and fat, also might be present in kidney, and may be detected in liver and spleen of LAM patients.  LAM could be sporadic (S-LAM) or associated with hamartoma syndrome tuberous sclerosis complex (TSC-LAM).  The disease affects 3-8 women per million in S-LAM.  At least 30% of women with TSC develop cystic changes in the lung consistent with LAM.  The risk of LAM in TSC patients, however, is age-related.  Thus, the prevalence of LAM by age 21 is 27% and 81% in TSC-LAM patients over 41 years old. Importantly, 63% of TSC patients develop LAM, and 13% die from LAM.  The mean onset of the disease is about 34-35 years, and patients registered with The LAM Foundation show a median survival of 29 years after onset of symptoms. Major advances have been made in inhibiting LAM cell growth by using allosteric inhibitors of mTOR activity such as sirolimus and everolimus, generally named rapalogs. These drugs slow down the disease progression by stabilizing the decline in lung function, but there is no cure for LAM.

 

Reynold A. Panettieri Jr., MD

Reynold A. Panettieri Jr., MD
Robert L. Mayock and David A. Cooper Professor of Medicine
Pulmonary, Allergy and Critical Care Division
Director, Airways Biology Initiative
University of Pennsylvania Perelman School of Medicine
Adjunct Professor, Wistar Institute
University of Pennsylvania Perelman School of Medicine
Member, ATS Board of Directors

Mary Harbaugh, PhD

Mary Harbaugh, PhD
Board Member
The LAM Foundation
Member, ATS Public Advisory Roundtable

 

ATS LAM PARTNER
The LAM Foundation

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The LAM Foundation urgently seeks safe and effective treatments, and ultimately a cure, for lymphangioleiomyomatosis (LAM) through advocacy and the funding of promising research. We are dedicated to serving the scientific, medical and patient communities by offering information, resources and a worldwide network of hope and support.