Rare Disease Report

New Guidelines for Diagnosing LAM Published Today

NOVEMBER 15, 2017
Mathew Shanley
Additional clinical practice guidelines were published this morning as it pertains to 4 specific questions related to diagnosing lymphangioleiomyomatosis (LAM) and the management of pneumothoraces in patients with it.

The new guidelines are a collaborative effort between the American Thoracic Society (ATS) and Japanese Respiratory Society (JRS), and complement previously published guidelines issued by the pair a year ago.

In patients with LAM, smooth muscle cells – most commonly in the lungs, lymphatic system and kidneys – begin to grow abnormally, leading to an accumulation of fluid in the chest and the development of tumors in vital organs. If not treated properly, lung function will worsen at nearly three times its normal rate, leading to complete respiratory failure.

The condition most frequently affects women between young adulthood and middle age, and there aren’t any treatments currently approved to slow or reverse progression of it.

Four recommendations were made by a 21-member multidisciplinary committee consisting of clinicians and scientists, each one having quality evidence to support it rated by the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach. They are as follows:

“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, Pulmonary Branch of the National Heart, Lung and Blood Institute in the official ATS press release. “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.”

Francis X. McCormack, MD, co-chair of the guideline committee, also commented, saying that the hope for the guidelines is that they “can help protect patients from inappropriate drug exposures.” Nishant Guputa, MD, first author of the guidelines noted that while the patient population and body of evidence are both small, the group “identified several research directions that would greatly strengthen future recommendations.”

The full guidelines writing committee included: Drs. Moss, McCormack and Gupta, as well as Geraldine A. Finlay, MD; Robert M. Kotloff, MD; Charlie Strange, MD; and Kevin C. Wilson, MD.

The guidelines can be found in the November 15 issue of the American Thoracic Society’s American Journal of Respiratory and Critical Care Medicine.

LAM currently impacts between 3 and 5 in every million people.

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