lung disease

New ATS Guidelines Address Rare Lung Disease

The American Thoracic Society (ATS) and the Japanese Respiratory Society (JRS) have released additional clinical practice guidelines regarding the diagnosis of lymphangioleiomyomatosis (LAM) and management of pneumothoraces in patients with LAM.

The new guidelines, which are published in the November 15 issue of the American Journal of Respiratory and Critical Care Medicine, supplement LAM guidelines that were previously issued by ATS and JRS in 2016.
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Approximately 5 of every 1 million women are affected by LAM, a systemic disease in which neoplastic smooth muscle-like cells arise from an unknown source, infiltrate the lung, and lead to cystic changes. As a result, lung function in these patients declines at 2 to 4 times the normal rate, and patients with the condition experience repeated lung collapses.

The following 4 recommendations for the diagnosis and management of LAM have been made by a 21-member multidisciplinary committee of clinicians and scientists, using the Grading of Recommendations, Assessment, Development, and Evaluation approach:

  • High-resolution computed tomography (HRCT) findings should not be used to make a clinical diagnosis of LAM in patients who have cystic changes in the lung characteristic of LAM on HRCT but have no other features of LAM.
  • A diagnostic approach including transbronchial lung biopsy prior to a surgical lung biopsy is suggested when a definitive diagnosis of LAM is necessary.
  • Rather than wait for a recurrent pneumothorax before intervening with a pleural symphysis procedure, it is suggested that patients with LAM should be offered ipsilateral pleurodesis after their first pneumothorax.
  • It is suggested that previous unilateral or bilateral pleural procedures should not be considered a contraindication to lung transplantation in patients with LAM.

“We continue to adjust our guidelines to keep pace with the evidence from clinical research,” said Joel Moss, MD, PhD, co-chair of the guideline committee and deputy chief of the Pulmonary Branch at the National Heart, Lung, and Blood Institute in Bethesda, Maryland. “Patients are individuals and clinicians look at their specific cases when making treatment decisions. Their awareness of the guidelines and of the level of confidence in the potential effects of any course of treatment informs a doctor’s decision making, without constraining it. That is the key benefit to patients.”

—Christina Vogt

Reference:

New guidelines issued for diagnosis and care of LAM, a rare lung disease [press release]. American Thoracic Society (ATS) and Japanese Respiratory Society (JRS). http://www.thoracic.org/about/newsroom/press-releases/journal/new-guidelines-issued-for-diagnosis-and-care-of-lam-a-rare-lung-disease.php. Accessed November 15, 2017.