LAM

LAM Week

LAM Week

Welcome Message

Welcome to Lymphangioleiomyomatosis (LAM) week at ATS 2017!  LAM is a rare, neoplastic, disorder. While LAM can affect many organs in the body, it most often causes a progressive and irreversible lung disease due to loss of lung function from the appearance of multiple cysts in the lung.  LAM is either a sporadic disorder (S-LAM), affecting women of childbearing age, or a lung manifestation of the inherited disease tuberous sclerosis complex (TSC-LAM).  Shortness of breath and fatigue often limit the physical activities of affected individuals and disrupt LAM patients’ lives, as well as cough, coughing up blood (hemoptysis) and spontaneous pneumothorax (lung collapse from the rupture of cysts).  Major advancements in research have led to the discoveries that mutations in genes named TSC1 or TSC2 are found in most patients with LAM, including those with S-LAM. 

Historically, surgical lung biopsy has been considered the gold standard procedure to establish a diagnosis.  However, recent discoveries allow a diagnosis of LAM to be made when characteristic findings are identified on complementary testing that includes high resolution computed tomography (HRCT)  along with either other classic clinical features (e.g., renal angiomyolipoma or chylous effusions), or  transbronchial biopsied lung tissue staining positive for HMB-45 or elevated VEGF-D levels in blood.

Although the pathobiology of LAM has yet to be fully elucidated, groundbreaking research has  confirmed that the mammalian target of rapamycin (mTOR) signaling pathway is the lynchpin of its pathogenesis. Even more exciting is the discovery that mTOR inhibitors, such as sirolimus or everolimus, slow LAM progression.

With the LAM Foundation, our PAR partner, we at ATS hope this week’s LAM-specific program provides patients, clinicians, and researches with the educational and scientific resources they seek.  We also hope this week highlights the LAM Foundation’s enduring commitment to support LAM patients, their families and friends, and the medical community as we move forward together toward a future with even better therapies, and ultimately a cure for LAM.  

 

Irina Petrache, MD

Irina Petrache, MD
Chief, Pulmonary, Critical Care and Sleep Medicine
Wollowick Chair in COPD Research
National Jewish Health
Professor, Department of Medicine, National Jewish Health
Professor, University of Colorado, Denver

Mary Harbaugh, PhD

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

Contributor: Amy Olson, MD, MSPH
National Jewish Health
Assistant Professor
Department of Medicine
Division of Pulmonary, Critical Care and Sleep Medicine
Member, American Thoracic Society

 

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.