Rare Lung Disease II

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Rare Lung Disease Week II

Rare Lung Disease Week II

Welcome Message

On behalf of the American Thoracic Society (ATS), and the Lymphangiomatosis and Gorham’s Disease Alliance (LGDA), a member of the ATS Public Advisory Roundtable, we would like to welcome you to Rare Lung Disease Week II.  This week, we feature generalized lymphatic anomaly (GLA), also known as lymphangiomatosis.  We will focus on its pulmonary complications; sometimes described as diffuse pulmonary lymphangiomatosis (DPL) or Kaposiform lymphangiomatosis. These are non-hereditary disorders, thought to result from a congenital lymphatic malformation, in which proliferation of lymphatic endothelial (vascular) cells leads to lung invasion, chylous (i.e., lymphatic, fatty) pleural and pericardial effusions (fluid collections, chylothorax, chylopericardium), and respiratory failure. The etiology of GLA is not known and there is no standard treatment, although some therapies have been reported to be successful.   A closely related disorder, Gorham-Stout Disease (also of a suspected congenital lymphatic malformation origin) which is characterized by penetration of the bone cortex can also have the above thoracic complications.

GLA is a rare disease with a 16% fatality rate reported in the literature that may be higher than 50% in patients with thoracic involvement. It affects children, who have a worse prognosis, more than adults. Yet, because health care providers are unlikely to see the disease in practice, the average time to diagnosis is greater than 6 years after symptom onset. Histopathologic misdiagnosis occurs in close to one half of cases, leading to further diagnostic delay and disease progression.

The ATS and LGDA are pleased to bring GLA to the attention of the public and the health care provider communities. It is essential that research funding be available to enhance our understanding of the etiology and treatment of this devastating disease. We invite you to join our webinar on August 5, 2015 at 3p EST.

 

Robin Gross, MD, FCCP

Robin Gross, MD, FCCP
Associate Professor
Division of Pulmonary, Critical Care and Sleep Medicine
Georgetown University
Member, ATS Board of Directors

Donna Appell, RN

Jack Kelly
President, Lymphangiomatosis & Gorham’s Disease Alliance
Member, ATS Public Advisory Roundtable

 

ATS Rare Lung Disease PARTNER
The LGD Alliance

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The mission of the LGD Alliance is to improve the care of patients with lymphangiomatosis and Gorham’s disease by promoting research that will identify effective treatments and ultimately a cure for these diseases. The Alliance is committed to providing support to members of the patient community and their families; education for the community, professionals, and the general public; and hope to those affected by these rare lymphatic malformations.